129 results match your criteria: "Paraneoplastic Autonomic Neuropathy"

Complications that occur during cancer therapy have emerged as a major contributor to the poor quality of life experienced by cancer patients as they live longer due to improved treatments. Many studies have investigated chemotherapy-induced peripheral neuropathy, but few have investigated the autonomic nervous system. Cardiovascular autonomic dysfunction (CAD) contributes to the distressing symptoms experienced by cancer patients, and it is also related to poor treatment outcomes.

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Article Synopsis
  • Chronic lymphoproliferative disorder of natural killer cells (CLPD-NK) is a rare disease with high NK cell counts that is often asymptomatic, but can lead to symptoms like fatigue, fever, and autoimmune diseases.
  • A 61-year-old male patient diagnosed with CLPD-NK experienced numbness and elevated protein levels in cerebrospinal fluid, indicating potential immunological issues, which were treated with steroids and immunoglobulin therapy.
  • Despite treatment alleviating some symptoms, the patient developed significant autonomic dysfunction, leading to issues like diarrhea and orthostatic hypotension, while tests for specific antibodies were negative, and a nerve biopsy suggested lymphocytic infiltration.
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The utility of the normal thin section skin biopsy in the assessment of systemic/extracutaneous disease and small fiber neuropathy.

Clin Dermatol

December 2024

New York- Presbyterian/Weill Cornell Medicine Department of Pathology and Laboratory Medicine, New York, NY, USA.

Article Synopsis
  • Various systemic diseases can show abnormalities on a normal skin biopsy, which can assist in making accurate diagnoses, especially for conditions like thrombotic microvascular diseases and dysautonomia syndromes.
  • A review of skin biopsy data revealed specific patterns and markers that are linked to certain diseases, including increased C5b-9 deposition and signaling changes related to interferon.
  • The findings emphasize that skin biopsies provide valuable insights into multi-organ diseases, highlighting their importance in clinical evaluations.
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Neurological immune-related adverse events (irAEs) associated with cancer treatment with immune checkpoint inhibitors (ICI) present diverse clinical characteristics. Neurological irAEs affect the peripheral nervous system and muscles more than they affect the central nervous system. Among the various subsets of peripheral neuropathies, polyradiculoneuropathy, which includes Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy, stands out as the most severe form, leading to significant muscle weakness.

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Paraneoplastic autonomic neuropathies and GI dysmotility.

Handb Clin Neurol

March 2024

Department of Neurology, UT Southwestern Medical Center, Dallas, TX, United States. Electronic address:

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.

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Paraneoplastic neuropathies and peripheral nerve hyperexcitability disorders.

Handb Clin Neurol

March 2024

Department of Neurology, Mayo Clinic, Rochester, MN, United States; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, United States. Electronic address:

Article Synopsis
  • Peripheral neuropathy is a significant reason patients are referred to neurologic clinics, with paraneoplastic neuropathies being a severe subgroup that can lead to high morbidity and mortality.
  • Symptoms often include weakness, sensory loss, autonomic dysfunction, severe constipation, and pain, and these neuropathies may be the first sign of underlying malignancy in patients.
  • Identifying specific antibodies can help target cancer investigations, as well as differentiate between various types of neuropathies, emphasizing the importance of testing for multiple antibodies simultaneously for better diagnostic sensitivity.
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  • Hodgkin lymphoma (HL) is a type of cancer that originates from B lymphocytes and can occasionally lead to neurological issues, either through direct invasion or as paraneoplastic syndromes.
  • The most common neurological syndrome linked to HL is paraneoplastic cerebellar degeneration, but others like limbic encephalitis and neuronopathy can also occur.
  • A case study highlighted a woman whose HL first showed symptoms of sensory and autonomic neuronopathy, where treatment for HL led to significant improvement in autonomic symptoms, though sensory symptoms showed only limited recovery.
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  • * A 57-year-old male patient diagnosed with papillary thyroid microcarcinoma presented with autonomic neuropathy and tested positive for anti-SOX1 antibodies.
  • * Following a total thyroidectomy, the patient's symptoms improved rapidly, and subsequent tests showed negative results for anti-SOX1 antibodies, indicating a potential link between thyroid cancer and PNS.
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  • In 2014, researchers identified a type of autoantibody, ITPR1-IgG/anti-Sj, linked to autoimmune cerebellar ataxia, prompting a review of its clinical characteristics and related findings.
  • The study analyzed existing literature and presented a new case of encephalitis associated with these antibodies, detailing severe cognitive and psychotic symptoms in a patient.
  • Findings revealed that ITPR1-IgG/anti-Sj affects various neurological conditions and is often associated with tumors; however, treatment yielded limited effectiveness in most patients.
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Morvan Syndrome (MoS) is an autoimmune disorder characterized by peripheral nerve hyperexcitability, autonomic dysfunction, and encephalopathy. We describe the case of a man with a history of thymoma diagnosed with a paraneoplastic MoS with a severe painful neuropathy refractory to immunoglobulins and steroids who had a dramatic and lasting response following treatment with rituximab. We also reviewed the clinical features, comorbidities, laboratory findings, treatment responses, relapses, and long-term outcomes of all published cases of MoS treated with rituximab.

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  • Paraneoplastic neurological syndrome (PNS) is a rare cancer-related complication that can impact various parts of the nervous system, causing diverse neurological symptoms, with certain antibody types frequently involved.* -
  • A 55-year-old woman with a history of breast cancer was found to have small-cell lung cancer and exhibited unusual neurological symptoms associated with both anti-Yo and anti-Hu antibodies, marking a rare case of concurrent paraneoplastic conditions.* -
  • This case highlights the need for increased awareness regarding the occurrence of multiple tumors and corresponding paraneoplastic antibodies in patients, as it may complicate diagnosis and treatment.*
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Movement disorders and neuropathies: overlaps and mimics in clinical practice.

J Neurol

September 2022

Clinical Neurology Unit, "Azienda Socio-Sanitaria Territoriale Santi Paolo E Carlo" and Department of Health Sciences, University of Milan, Via Antonio di Rudinì 8, 20142, Milan, Italy.

Article Synopsis
  • Movement disorders and peripheral neuropathies frequently occur together in the population, often due to common causes like diabetes and Parkinson's disease (PD), but there's evidence suggesting PD can also trigger polyneuropathy.
  • Some peripheral neuropathies may lead to movement disorders like tremor, hinting that central mechanisms might affect both conditions over time.
  • Identifying the combination of these disorders requires thorough examinations and testing, particularly in cases with a family history or early onset, focusing on genetic and treatable causes like autoimmune or nutritional factors.
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Article Synopsis
  • - Paraneoplastic syndromes, affecting about 0.1% of cancer patients, are more common in certain tumors like Small Cell Lung Cancer (SCLC) and Thymoma, as they trigger autoimmune responses against nervous system factors produced by the tumors.
  • - The article discusses the relationship between immunotherapy and paraneoplastic neurological syndromes (PNS), highlighting that the use of immunotherapy in SCLC may lead to an increase in autoimmune neurological symptoms.
  • - The authors share their experience with three patients at the Istituto Nazionale Tumori in Milan, noting that typical cancer treatments have little effect on neurological deficits caused by PNS, indicating a need for more research to improve patient care.
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Video Representation of Dopamine-Responsive Multiple System Atrophy Cerebellar Type.

Am J Case Rep

November 2021

Department of Neurology, College of Medicine and Life Sciences, University of Toledo, Toledo, OH, USA.

Article Synopsis
  • * A case study of a 61-year-old man with MSA-C showed significant improvement in motor function after starting carbidopa/levodopa therapy, despite generally low expectations for treatment response in this condition.
  • * The findings emphasize the variability in treatment responses among MSA patients, suggesting the need for further research into effective therapies for MSA and Parkinson's disease.
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Introduction: Autoimmune neuropathies have diverse presentations and underlying immune mechanisms. Demonstration of efficacy of therapeutic agents that inhibit the complement cascade would confirm the role of complement activation.

Areas Covered: A review of the pathophysiology of the autoimmune neuropathies, to identify those that are likely to be complement mediated.

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Background: Paraneoplastic neurological syndromes (PNSs) are broad-spectrum disorders that can affect any part of the nervous system varying in core symptoms. Onconeural antibodies, including Hu, Yo, Ri, anti-CV2, amphiphysin, Ma2, and Tr are well-characterized and commonly used for the diagnosis of definite PNS. Generally, anti-CV2 antibodies have usually been associated with cerebellar ataxia, chorea, peripheral and autonomic neuropathies, myelopathy, optic neuritis, and retinitis.

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Paraneoplastic Neuropathies: What's New Since the 2004 Recommended Diagnostic Criteria.

Front Neurol

October 2021

Neurology Unit, Ospedale S Chiara, Azienda Provinciale per i Servizi Sanitari (APSS), Trento, Italy.

Article Synopsis
  • The 2004 diagnostic criteria by the PNS Euronetwork established a classification system for paraneoplastic neurological syndromes (PNS), identifying subacute sensory neuronopathy (SSN) as the most common peripheral PNS, while other types like sensory polyneuropathy and focal nerve lesions were less prevalent.
  • Since then, understanding of onconeural antibodies and their relation to different tumors has expanded, revealing more about mechanisms behind neuropathies in conditions like lymphoma and multiple myeloma.
  • The range of paraneoplastic neuropathies has broadened to include motor and small fiber neuropathies, with new complications arising from modern cancer treatments, necessitating assessments between PNS and chemotherapy-induced peripheral neuropathy (C
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Purpose: Peripheral autonomic neuropathy, including enteric neuropathy, may be subtle and unrecognized for several years. Diagnosis of enteric neuropathy demands complicated examinations such as full-thickness bowel biopsy. We hypothesized that knowledge about simultaneous occurrence of different types of neuropathy would lead to faster recognition and diagnosis of autonomic/enteric neuropathy.

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Gastrointestinal dysfunction in neuroinflammatory diseases: Multiple sclerosis, neuromyelitis optica, acute autonomic ganglionopathy and related conditions.

Auton Neurosci

May 2021

Neurology, Internal Medicine, Sakura Medical Center, Toho University, 564-1 Shimoshizu, Sakura 285-8741, Japan. Electronic address:

Disorders of the nervous system can produce a variety of gastrointestinal (GI) dysfunctions. Among these, lesions in various brain structures can cause appetite loss (hypothalamus), decreased peristalsis (presumably the basal ganglia, pontine defecation center/Barrington's nucleus), decreased abdominal strain (presumably parabrachial nucleus/Kolliker-Fuse nucleus) and hiccupping and vomiting (area postrema/dorsal vagal complex). In addition, decreased peristalsis with/without loss of bowel sensation can be caused by lesions of the spinal long tracts and the intermediolateral nucleus or of the peripheral nerves and myenteric plexus.

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Chemotherapy and Radiation-Associated Cardiac Autonomic Dysfunction.

Curr Oncol Rep

January 2021

UCLA Cardio-Oncology Program, Division of Cardiology, Department of Medicine, University of California at Los Angeles, 100 Medical Plaza, Suite 630, Los Angeles, CA, 90095, USA.

Purpose Of Review: Cardiovascular autonomic dysfunction (AD) among cancer survivors is increasingly being recognized. However, the mechanisms and incidence are poorly understood. In this review, the clinical features, diagnostic modalities, proposed mechanisms, and currently available treatments of cardiovascular AD in cancer survivors are described.

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We describe the case of a 74-year-old fit and healthy man who developed a profound sleep disorder characterized by mid-day hypersomnia and debilitating insomnia. A wide range of therapies, including a large number of stimulants and hypnotics with multiple different mechanisms of action, failed to improve his condition. Trials with oral prosthetic devices and a wide range of face masks with positive pressure assistance and multiple continuous positive airway pressure (CPAP) titration studies failed to help.

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The peripheral nervous system (PNS) relays messages between the central nervous system (brain and spinal cord) and the body. Despite this critical role and widespread distribution, the PNS is often overlooked when investigating disease in diagnostic and experimental pathology. This review highlights key features of neuroanatomy and physiology of the somatic and autonomic PNS, and appropriate PNS sampling and processing techniques.

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Autonomic neuropathies.

Muscle Nerve

January 2021

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

Autonomic neuropathies represent a complex group of disorders that preferentially target autonomic fibers and can be classified as either acute/subacute or chronic in onset. Acute-onset autonomic neuropathies manifest with such conditions as paraneoplastic syndromes, Guillain-Barre syndrome, Sjögren syndrome, infection, or toxins/chemotherapy. When the presentation is acute, immune-mediated, and without a secondary cause, autoimmune autonomic ganglionopathy is likely, and should be considered for immunotherapy.

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Stiff Person Syndrome and Acetylcholine Receptor Ganglionic Neuronal Antibodies.

Case Rep Neurol

January 2020

Department of Neurosurgery, Instituto Mexicano de Neurociencias, Huixquilucan, Mexico.

The association between stiff person syndrome and paraneoplastic syndromes has been described, linking intracellular or extracellular antibodies. We describe the case of a 64-year-old woman with stiff person syndrome and positivity for acetylcholine receptor ganglionic neuronal antibodies, which can also be seen in muscular hyperexcitability conditions, as well as other paraneoplastic syndromes. The relevance of this report is the possible direct elevation of this antibody due to a direct immunological cause.

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