108 results match your criteria Paraneoplastic Autonomic Neuropathy

Refractory Morvan syndrome responsive to rituximab: A case report and review of the literature.

Neuromuscul Disord 2022 Jun 2. Epub 2022 Jun 2.

American University of Beirut Medical Center, Lebanon.

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. Read More

View Article and Full-Text PDF

Paraneoplastic neurological syndrome with positive anti-Hu and anti-Yo antibodies: A case report.

World J Clin Cases 2022 May;10(13):4190-4195

Department of Neurology, Lanzhou University Second Hospital, Lanzhou 730000, Gansu Province, China.

Background: Paraneoplastic neurological syndrome (PNS) is a rare complication in patients with cancer. PNS can affect the central, peripheral, autonomic nervous system, neuromuscular junction, or muscles and cause various neurological symptoms. Anti-Yo antibody-positive neurological paraneoplasms and anti-Hu antibody-positive neurological paraneoplasms are common, but coexistence of both types has not been described in the literature. Read More

View Article and Full-Text PDF

Movement disorders and neuropathies: overlaps and mimics in clinical practice.

J Neurol 2022 Jun 3. Epub 2022 Jun 3.

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.

Movement disorders as well as peripheral neuropathies are extremely frequent in the general population; therefore, it is not uncommon to encounter patients with both these conditions. Often, the coexistence is coincidental, due to the high incidence of common causes of peripheral neuropathy, such as diabetes and other age-related disorders, as well as of Parkinson disease (PD), which has a typical late onset. Nonetheless, there is broad evidence that PD patients may commonly develop a sensory and/or autonomic polyneuropathy, triggered by intrinsic and/or extrinsic mechanisms. Read More

View Article and Full-Text PDF

Paraneoplastic neurological syndromes in patients affected by SCLC: a case series.

Tumori 2022 Mar 8:3008916221079663. Epub 2022 Mar 8.

Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

Paraneoplastic syndromes occur in about 0.1% of patients affected by neoplastic diseases. In some types of tumors, such as Small Cell Lung Cancer (SCLC), Thymoma, and particular forms of Plasmacytoma, the association with Paraneoplastic Neurological Syndromes (PNS) is much more frequent. Read More

View Article and Full-Text PDF

Video Representation of Dopamine-Responsive Multiple System Atrophy Cerebellar Type.

Am J Case Rep 2021 Nov 15;22:e933995. Epub 2021 Nov 15.

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

BACKGROUND Multiple system atrophy cerebellar type (MSA-C) is a subtype of MSA that presents with predominant ataxia along with lesser signs of parkinsonism and autonomic dysfunction. Previous studies have shown benefits from carbidopa/levodopa therapy for the MSA parkinsonian subtype but few studies have focused on the MSA-C subtype. We present a video case of MSA-C that demonstrated significant improvement with carbidopa/levodopa therapy. Read More

View Article and Full-Text PDF
November 2021

Immune mechanisms, the role of complement, and related therapies in autoimmune neuropathies.

Norman Latov

Expert Rev Clin Immunol 2021 12 23;17(12):1269-1281. Epub 2021 Nov 23.

Department of Neurology, Weill Cornell Medical College, New York, USA.

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. Read More

View Article and Full-Text PDF
December 2021

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

Front Neurol 2021 1;12:706169. Epub 2021 Oct 1.

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

The diagnostic criteria published by the PNS (Paraneoplastic Neurological Syndromes) Euronetwork in 2004 provided a useful classification of PNS, including paraneoplastic neuropathies. Subacute sensory neuronopathy (SSN) was the most frequently observed peripheral PNS, whereas other forms of neuropathy, as sensory polyneuropathy, sensorimotor polyneuropathy, demyelinating neuropathies, autonomic neuropathies, and focal nerve or plexus lesions, were less frequent. At the time of publication, the main focus was on onconeural antibodies, but knowledge regarding the mechanisms has since expanded. Read More

View Article and Full-Text PDF
October 2021

Diseases which cause generalized peripheral neuropathy: a systematic review.

Scand J Gastroenterol 2021 09 2;56(9):1000-1010. Epub 2021 Jul 2.

Department of Internal Medicine, Skåne University Hospital, Malmö, Sweden.

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. Read More

View Article and Full-Text PDF
September 2021

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

Ryuji Sakakibara

Auton Neurosci 2021 05 13;232:102795. Epub 2021 Mar 13.

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. Read More

View Article and Full-Text PDF

Chemotherapy and Radiation-Associated Cardiac Autonomic Dysfunction.

Curr Oncol Rep 2021 01 8;23(2):14. Epub 2021 Jan 8.

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. Read More

View Article and Full-Text PDF
January 2021

Glutamic Acid Decarboxylase (GAD-65) Autoimmunity Associated With Profound Daytime Hypersomnia, Nighttime Insomnia, Mild Autonomic Neuropathy and Axonal Sensori-Motor Polyneuropathy: A Case Report on a New Phenotype.

Cureus 2020 Oct 23;12(10):e11112. Epub 2020 Oct 23.

Neurology, Flowers Medical Group, Dothan, USA.

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. Read More

View Article and Full-Text PDF
October 2020

Comparative Pathology of the Peripheral Nervous System.

Vet Pathol 2021 01 5;58(1):10-33. Epub 2020 Oct 5.

GEMpath Inc, Longmont, CO, USA.

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. Read More

View Article and Full-Text PDF
January 2021

Autonomic neuropathies.

Muscle Nerve 2021 01 14;63(1):10-21. Epub 2020 Sep 14.

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. Read More

View Article and Full-Text PDF
January 2021

Subacute-onset paraneoplastic autonomic neuropathy associated with prostate cancer.

Clin Auton Res 2020 12 20;30(6):579-580. Epub 2020 Aug 20.

Neurology Unit, S. Orsola-Malpighi University Hospital, Via Giuseppe Massarenti 9, 40139, Bologna, IT, Italy.

View Article and Full-Text PDF
December 2020

Stiff Person Syndrome and Acetylcholine Receptor Ganglionic Neuronal Antibodies.

Case Rep Neurol 2020 Jan-Apr;12(1):24-26. Epub 2020 Jan 15.

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. Read More

View Article and Full-Text PDF
January 2020

Autonomic Peripheral Neuropathy.

Roy Freeman

Continuum (Minneap Minn) 2020 Feb;26(1):58-71

Purpose Of Review: This article provides a summary of the autonomic neuropathies, including neuropathies associated with diabetes mellitus, neuropathies due to amyloid deposition, immune-mediated autonomic neuropathies (including those associated with a paraneoplastic syndrome), inherited autonomic neuropathies, and toxic autonomic neuropathies. The presenting features, diagnostic investigations, and natural history of these neuropathies are discussed.

Recent Findings: Recent findings in autonomic peripheral neuropathy include data on the epidemiology and atypical presentations of diabetic autonomic neuropathy, treatment-induced neuropathy of diabetes mellitus, the presentation of immune-mediated neuropathies, and advances in hereditary neuropathy associated with amyloidosis and other hereditary neuropathies. Read More

View Article and Full-Text PDF
February 2020

Peripheral neuropathy in hematologic malignancies - Past, present and future.

Blood Rev 2020 09 17;43:100653. Epub 2020 Jan 17.

Brain and Mind Centre, School of Medical Sciences, Faculty of Medicine and Health, The University of Sydney, Camperdown, Australia. Electronic address:

Neurotoxic treatments (including proteasome inhibitors, immunomodulatory drugs and vinca-alkaloids) are often used in the treatment of hematologic malignancy. Peripheral neuropathy can be part of a paraneoplastic syndrome accompanying the disease but more commonly is a consequence of treatment with neurotoxic therapies, and produces sensory, motor, autonomic nerve dysfunction or a combination, leading to pain, loss of sensation and functional disability. This review provides an update on peripheral neuropathy in hematologic malignancy, including risk factors, mechanisms and treatment options. Read More

View Article and Full-Text PDF
September 2020

Autonomic dysfunction is frequent and disabling in non-paraneoplastic sensory neuronopathies.

J Neurol Sci 2019 Jul 16;402:111-117. Epub 2019 May 16.

Neurology Department, Neuromuscular Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil. Electronic address:

Introduction: Sensory neuronopathies (SN) are characterized by asymmetric non-length dependent sensory deficits and sensory ataxia. Autonomic dysfunction in SN was not yet evaluated regarding its frequency, characteristics and relationship to sensory deficits. To address these issues, we performed a comprehensive clinical and neurophysiological evaluation of a large cohort of patients with non-paraneoplastic SN (np-SN). Read More

View Article and Full-Text PDF

Autoimmune autonomic neuropathies and ganglionopathies: epidemiology, pathophysiology, and therapeutic advances.

Clin Auton Res 2019 06 15;29(3):277-288. Epub 2019 May 15.

Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd. MC 9368, Dallas, TX, 75390, USA.

Autonomic disorders can be the result of autoimmunity. The classic, well-characterized example is autoimmune autonomic ganglionopathy (AAG), in which antibodies against the ganglionic nicotinic acetylcholine receptor impair autonomic transmission, causing autonomic failure, which responds to immunotherapy. However, a number of other autoimmune disorders cause autonomic failure through a variety of mechanisms. Read More

View Article and Full-Text PDF

Neurological autoimmune disorders with prominent gastrointestinal manifestations: A review of presentation, evaluation, and treatment.

Neurogastroenterol Motil 2019 10 23;31(10):e13611. Epub 2019 Apr 23.

Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas.

Background: The identification of autoantibodies directed against neuronal antigens has led to the recognition of a wide spectrum of neurological autoimmune disorders (NAD). With timely recognition and treatment, many patients with NAD see rapid improvement. Symptoms associated with NAD can be diverse and are determined by the regions of the nervous system affected. Read More

View Article and Full-Text PDF
October 2019

Hypotension as a symptom of autonomic neuropathy in patients with advanced malignancies.

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2019 Dec 11;163(4):331-334. Epub 2018 Dec 11.

Department of Oncology, Faculty Hospital Trencin, Legionarska 28, Trencin, Slovak Republic.

Aims: Hypotension can be a symptom of paraneoplastic autonomic neuropathy (PAN). Onconeural antibodies (OA) provide strong evidence for the paraneoplastic origin of neurological syndromes. Our goal was to assess the frequency of PAN among patients with advanced malignancies and hypotension using OA. Read More

View Article and Full-Text PDF
December 2019

Integrated treatment for autonomic paraneoplastic syndrome improves performance status in a patient with small lung cell carcinoma: a case report.

BMC Neurol 2018 Nov 10;18(1):189. Epub 2018 Nov 10.

Department of Neurology, Aomori Prefectural Central Hospital, 2-1-1 Higashi-Tsukurimichi, Aomori, 030-8551, Japan.

Background: Paraneoplastic neurological syndromes (PNS) are rare disorders associated with cancer and are believed to be immune mediated. Patients with autonomic PNS suffer from variable combinations of parasympathetic and sympathetic failure. Autonomic PNS are usually associated with other PNS, such as encephalomyelitis and sensory neuropathy; however, autonomic symptoms may rarely manifest as PNS symptoms. Read More

View Article and Full-Text PDF
November 2018

Classical Hodgkin Lymphoma Presenting with Severe, Recurrent Hypothermic Episodes.

Case Rep Hematol 2018 25;2018:3726593. Epub 2018 Sep 25.

Department of Hematology, Aarhus University Hospital, Tage-Hansens Gade 2, DK-8000 Aarhus, Denmark.

We report a case presenting with recurrent episodes of severe hypothermia preceding the diagnosis of Hodgkin lymphoma. The episodes of hypothermia were accompanied by other symptoms of autonomic dysfunction, mainly hypotension, which could be caused by autonomic neuropathy as part of a paraneoplastic syndrome. In comparison with previous reports describing an association between the presence of hypothermia and an adverse outcome, the present patient has responded well to lymphoma-specific treatment and is currently in an ongoing complete remission. Read More

View Article and Full-Text PDF
September 2018

Acute Autonomic Symptoms with Anti-myelin-associated Glycoprotein Neuropathy as a Presentation of Small B Cell Lymphoma: A Case Report and Literature Review.

Cureus 2018 Aug 6;10(8):e3105. Epub 2018 Aug 6.

Neurology, University of Missouri, Columbia, USA.

The association of symmetrical distal sensorimotor polyneuropathy with anti-myelin-associated glycoprotein antibodies (MAG) has been well established. Although autonomic symptoms are uncommon with anti-MAG antibody neuropathy (MAN). We are presenting an unusual case, who developed acute onset urinary retention, orthostatic hypotension, bradycardia and was found to have MAN. Read More

View Article and Full-Text PDF

[Transthyretin Familial Amyloid Polyneuropathy - Disease Profile of a Multisystem Disorder].

Dtsch Med Wochenschr 2018 Mar 15;143(6):427-430. Epub 2018 Mar 15.

Transthyretin-related Familial Amyloid Polyneuropathy (ATTR Amyloidosis, former FAP, here called TTR-FAP) is a rare, progressive autosomal dominant inherited amyloid disease ending fatal within 5 - 15 years after final diagnosis. TTR-FAP is caused by mutations of transthyretin (TTR), which forms amyloid fibrils affecting peripheral and autonomic nerves, the heart and other organs. Due to the phenotypic heterogeneity and partly not specific enough clinical symptoms, diagnosis of TTR-FAP can be complicated. Read More

View Article and Full-Text PDF

Neurologic Complications of Lymphoma, Leukemia, and Paraproteinemias.

Continuum (Minneap Minn) 2017 06;23(3, Neurology of Systemic Disease):669-690

Purpose Of Review: This article reviews the spectrum of neurologic complications associated with lymphoma, leukemia, and paraproteinemic disorders. While leptomeningeal metastasis is the most common complication of lymphoma and leukemia and peripheral neuropathy is the most common complication of paraproteinemic disorders, clinicians need to be familiar with the diverse neurologic complications of these disorders.

Recent Findings: Lymphomatous nervous system involvement can be difficult to diagnose, especially when it is the presenting symptom. Read More

View Article and Full-Text PDF

Inositol 1,4,5-trisphosphate receptor type 1 autoantibodies in paraneoplastic and non-paraneoplastic peripheral neuropathy.

J Neuroinflammation 2016 10 24;13(1):278. Epub 2016 Oct 24.

Department of Neurology, Medical Faculty, Heinrich Heine University, Moorenstraße 5, 40225, Düsseldorf, Germany.

Background: Recently, we described a novel autoantibody, anti-Sj/ITPR1-IgG, that targets the inositol 1,4,5-trisphosphate receptor type 1 (ITPR1) in patients with cerebellar ataxia. However, ITPR1 is expressed not only by Purkinje cells but also in the anterior horn of the spinal cord, in the substantia gelatinosa and in the motor, sensory (including the dorsal root ganglia) and autonomic peripheral nervous system, suggesting that the clinical spectrum associated with autoimmunity to ITPR1 may be broader than initially thought. Here we report on serum autoantibodies to ITPR1 (up to 1:15,000) in three patients with (radiculo)polyneuropathy, which in two cases was associated with cancer (ITPR1-expressing adenocarcinoma of the lung, multiple myeloma), suggesting a paraneoplastic aetiology. Read More

View Article and Full-Text PDF
October 2016

Is peripheral paraneoplastic neurological syndrome possible in primary brain tumors?

Brain Behav 2016 06 27;6(6):e00465. Epub 2016 Apr 27.

Department of Neurology Wroclaw Medical University Borowska 213 51-669 Wroclaw Poland.

Introduction: Systemic malignant diseases cause the induction of autoimmunity, for example, paraneoplastic syndromes. There are no proofs of paraneoplastic syndromes in primary brain tumors. The aim of the study was to evaluate the involvement of the peripheral nervous system, together with an assessment of onconeuronal and antineural antibodies as indicators of humoral immune response against nervous system in patients with primary brain tumors. Read More

View Article and Full-Text PDF

Autoimmune autonomic disorders.

Handb Clin Neurol 2016 ;133:405-16

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

Autoimmune autonomic disorders occur because of an immune response directed against sympathetic, parasympathetic, and enteric ganglia, autonomic nerves, or central autonomic pathways. In general, peripheral autoimmune disorders manifest with either generalized or restricted autonomic failure, whereas central autoimmune disorders manifest primarily with autonomic hyperactivity. Some autonomic disorders are generalized, and others are limited in their anatomic extent, e. Read More

View Article and Full-Text PDF
February 2017

Atypical case of Morvan's syndrome.

J Clin Neurosci 2016 Mar 6;25:132-4. Epub 2015 Nov 6.

Department of Neurology, Rabin Medical Center, Beilinson Campus 49100, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Morvan's syndrome is a rare neurological condition characterized by the combination of neuromyotonia, autonomic instability and encephalopathy, associated with auto-antibodies against voltage-gated potassium channels. We report a patient with an initial presentation suggestive of typical Guillain-Barré syndrome (GBS), who later developed clinical and laboratory features compatible with Morvan's syndrome. Several months after resolution of the neurological symptoms, as well as disappearance of the characteristic anti-leucine-rich, glioma inactivated 1 (anti-LGI1) antibodies, the patient presented with episodes of fever of unknown origin, during which the antibodies became positive again, suggesting the possibility of a relapse. Read More

View Article and Full-Text PDF