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https://www.readbyqxmd.com/read/29742813/mutated-cancer-autoantigen-implicated-cause-of-paraneoplastic-myasthenia-gravis
#1
Anastasia Zekeridou, Guy E Griesmann, Vanda A Lennon
INTRODUCTION: Anti-tumor immune responses are postulated to initiate paraneoplastic neurological disorders when proteins normally restricted to neural cells are expressed as oncoproteins. Mutated oncopeptides could bypass self-tolerant T-cells to activate cytotoxic effector T-lymphocytes and requisite helper T-lymphocytes to stimulate autoantibody production by B-lymphocytes. METHODS: We investigated muscle-type nicotinic acetylcholine receptor (AChR) antigen expression at transcriptional and protein levels in a small-cell lung cancer line (SCLC) established from a patient with AChR-IgG-positive myasthenia gravis...
May 9, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29718880/various-clinical-features-of-patients-with-anti-hu-associated-paraneoplastic-neurological-syndromes-an-observational-study
#2
Jia Li, Weihong Lin
To describe and analyze the clinical features and prognosis of patients with anti-Hu associated paraneoplastic neurological syndromes (PNS).The symptoms, MRI findings, cerebrospinal fluid (CSF) changes, electroencephalogram (EEG) characteristics and prognoses of 9 well-diagnosed anti-Hu associated PNS patients were analyzed.The study enrolled 6 female and 3 male patients. Three patients presented with vertigo and 6 patients exhibited a depressed mood, numbness of the lower limbs, generalized pains, seizures, mental disturbances, and a temporary unilateral hand tremor on initial presentation...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29717222/paraneoplastic-limbic-encephalitis-associated-with-lung-cancer
#3
Kaini Shen, Yan Xu, Hongzhi Guan, Wei Zhong, Minjiang Chen, Jing Zhao, Longyun Li, Mengzhao Wang
Paraneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in lung cancer patients. We retrospectively investigated the clinical characteristics, treatment responses, and prognoses in 16 PLE patients who were subsequently diagnosed with lung cancer. Fifteen patients initially presented with disturbance of consciousness, 13 with disorientation, and 12 with seizures. Thirteen patients had autoantibodies, including eight with gamma aminobutyric acid B receptor (GABAB R) antibodies and eight with Hu antibodies...
May 1, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29607578/anti-hu-antibody-mediated-limbic-encephalitis-associated-with-cervical-cancer-a-case-report
#4
Kyong Jin Shin, Yong Il Ji
A 55-year-old postmenopausal and multiparous woman presented to our department with recent memory disturbances associated with rapidly progressive positive Anti-Hu antibodies. She was subsequently diagnosed with anti-Hu antibody-related paraneoplastic limbic encephalitis. Clinical examination and imaging studies revealed a bulky cervical tumor with both parametrial and vaginal fornix extension; biopsy confirmed the tumor as cervical squamous cell carcinoma. In this case, we encountered a patient with anti-Hu-mediated paraneoplastic limbic encephalitis with a subsequent diagnosis of cervical cancer...
April 2, 2018: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/29561731/paraneoplastic-movement-disorders
#5
Karolina Popławska-Domaszewicz, Jolanta Florczak-Wyspiańska, Wojciech Kozubski, Sławomir Michalak
Paraneoplastic movement disorders are rare, autoimmune-mediated, nonmetastatic complications of malignant neoplasms. Common paraneoplastic movement disorders include paraneoplastic chorea, dystonia, cerebellar degeneration, different types of encephalitis, opsoclonus-myoclonus syndrome, stiff person syndrome, and neuromyotonia. Syndromes usually develop before tumor diagnosis, have subacute onset, and are associated with serum or cerebrospinal fluid antibodies. Two types of antibodies can be distinguished: antibodies against nuclear and cytoplasmic neuronal antigens (anti-Hu, anti-Ri, anti-Yo, anti-Ma, anti-CV2/CRMP5, anti-Gephrin, and anti-GABATRAP) and antibodies recently identified against cell surface and synaptic proteins (anti-NMDAR, anti-LGI1, and anti-Caspr2)...
March 21, 2018: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/29416500/msa-mimic-rare-occurrence-of-anti-hu-autonomic-failure-and-thymoma-in-a-patient-with-parkinsonism-case-report-and-literature-review
#6
Vito A G Ricigliano, Barbara Fossati, Lorenzo Saraceno, Michele Cavalli, Elena Bazzigaluppi, Giovanni Meola
Thymoma is a tumor originating from thymic gland, frequently manifesting with paraneoplastic neurological disorders. Its association with paraneoplastic dysautonomia is relatively uncommon. Here, we describe the challenging case of a 71 year-old female who developed subacute autonomic failure with digestive pseudo-obstruction, dysphagia, urinary tract dysfunction and orthostatic hypotension complicating an underlying extrapyramidal syndrome that had started 3 months before hospital admission. Autonomic symptoms had 2-month course and acutely worsened just before and during hospitalization...
2018: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29332416/-autoimmune-encephalitis-possibilities-in-the-laboratory-investigation
#7
Katalin Böröcz, Zsófia Hayden, Viktória Mészáros, Zsuzsanna Csizmadia, Kornélia Farkas, Zoltán Kellermayer, Péter Balogh, Ferenc Nagy, Tímea Berki
INTRODUCTION: The role of autoimmune responses against central nervous system (CNS) antigens in encephalitis presenting with non-classified neurologic or psychiatric symptoms has been appreciated in the past decade. Paraneoplastic limbic encephalitis has a poor prognosis and is most commonly associated with lung, ovarium, and testicular neoplasms, leading to immune reactions against intracellular antigens (anti-Hu/ANNA1, anti-Ri/ANNA2, anti-CV2/CRMP5 and anti-Ma2/Ta). In contrast, the recently described autoimmune encephalitis subtypes present with a broad spectrum of symptoms, respond to autoimmune therapies well and usually associate with autoantibodies against neuronal cell surface receptors (NMDAR, GABAB R, AMPAR) or synaptic proteins (LGI1, CASPR2)...
January 2018: Orvosi Hetilap
https://www.readbyqxmd.com/read/29318181/neuroimmunology-of-oms-and-anna-1-anti-hu-paraneoplastic-syndromes-in-a-child-with-neuroblastoma
#8
Michael R Pranzatelli, Nathan R McGee
No abstract text is available yet for this article.
March 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29287596/gamma-aminobutyric-acid-b-limbic-encephalitis-and-asystolic-cardiac-arrest-a-case-report
#9
Christopher A Ovens, Angelo Jayamanne, Andrew Duggins
BACKGROUND: Gamma-aminobutyric acid-B receptor autoantibodies are becoming an increasingly recognized contributor to the spectrum of autoimmune limbic encephalitis. They are classically associated with seizures and behavioral disturbance, and may coexist with other autoantibodies. Many are paraneoplastic, most commonly associated with small cell lung cancer. Until now there have been no reports of cardiac dysrhythmias in these patients. CASE PRESENTATION: A 65-year-old Caucasian man presented with multiple seizures, dysarthria and behavioral disturbance of unclear etiology, with associated asystolic cardiac arrest...
December 29, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29200116/paraneoplastic-disorders
#10
Eric Lancaster
PURPOSE OF REVIEW: Paraneoplastic neurologic syndromes target specific areas of the nervous system with pathogenic autoantibodies or T-cell responses. Each syndrome conveys a risk of particular tumors. Expanded paraneoplastic antibody testing has led to improved diagnosis but created challenges involving appropriate interpretation of test results. RECENT FINDINGS: Peripheral nervous system paraneoplastic disorders such as myasthenia gravis and Lambert-Eaton myasthenic syndrome involve pathogenic autoantibodies...
December 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29158368/anti-hu-associated-autoimmune-limbic-encephalitis-in-a-patient-with-pd-1-inhibitor-responsive-myxoid-chondrosarcoma
#11
Kyriakos P Papadopoulos, Rebecca S Romero, Gabriela Gonzalez, James E Dix, Israel Lowy, Matthew Fury
Autoimmune encephalitis is an uncommon complication of immune checkpoint inhibitor therapy. This article reports a case of fatal anti-Hu-associated autoimmune limbic encephalitis presenting within 8 weeks following anti-PD1 therapy in a patient with myxoid chondrosarcoma and pre-existing anti-Hu antibodies. Although tumor reduction occurred in response to PD-1 inhibitor therapy, the patient had a rapidly progressive decline in neurologic function despite initial stabilization with immunosuppression. Considering the increasing use of immune checkpoint inhibitors for the treatment of various malignancies, an increase in the occurrence of neurologic adverse events is likely, requiring prompt intervention and enhanced pharmacovigilance in malignancies associated with onconeuronal antibodies...
January 2018: Oncologist
https://www.readbyqxmd.com/read/29103425/cancer-and-autoimmunity-paraneoplastic-neurological-disorders-associated-with-neuroblastic-tumors
#12
Wendy G Mitchell, Franz Blaes
Cancer and autoimmunity come together in paraneoplastic syndromes (PNS), which reflect the remote, not direct, effects of cancer. In the pediatric population, a variety of PNS have been described, but the most common of these rare disorders are instigated by neuroblastic tumors, such as neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The main pediatric-onset neurological PNS are ROHHAD syndrome, anti-ANNA1 (anti-Hu), and opsoclonus-myoclonus syndrome. They manifest distinctive neurological features, which aid the diagnosis, though under-recognition still poses serious challenges and risks...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29078851/acquired-hemophilia-and-anti-hu-paraneoplastic-neurologic-syndrome-in-small-cell-lung-cancer
#13
Efi Aggelopoulou, Gerasimos Evangelatos, Konstantina Tzavida, Vasiliki Koulouri, Nikolaos Lazaridis
No abstract text is available yet for this article.
October 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28948076/peripheral-neuropathy-in-limbic-encephalitis-with-anti-glutamate-receptor-antibodies-case-report-and-systematic-literature-review
#14
Yi-Chia Wei, Chin-Chang Huang, Chi-Hung Liu, Hung-Chou Kuo, Jainn-Jim Lin
INTRODUCTION: Autoantibodies to the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor and N-methyl-d-aspartate (NMDA) receptor are known to be the causes of autoimmune encephalitis particularly limbic encephalitis. The involvement of the peripheral nervous system is rarely reported. METHODS: We analyzed the serial nerve conduction studies of a previously reported case of anti-AMPA receptor encephalitis, who was presented with conscious disturbance and quadriplegia...
September 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28883237/chronic-intestinal-pseudo-obstruction-and-orthostatic-hypotension-associated-with-small-cell-lung-cancer-that-improved-with-tumor-reduction-after-chemoradiotherapy
#15
Yusuke Izumi, Takeshi Masuda, Yasushi Horimasu, Taku Nakashima, Shintaro Miyamoto, Hiroshi Iwamoto, Kazunori Fujitaka, Hironobu Hamada, Noboru Hattori
Chronic intestinal pseudo-obstruction (CIPO) is a rare disease with symptoms of ileus without obstruction. Most cases of CIPO are idiopathic, and CIPO as a paraneoplastic neurological syndrome (PNS) associated with small cell lung cancer (SCLC) is rare. A 63-year-old man was diagnosed with functional ileus and confined to bed due to orthostatic hypotension. Chest computed tomography revealed a right hilar mass suspected of being lung cancer. Based on detailed examinations, he was diagnosed with limited-stage SCLC...
October 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28878663/subacute-cerebellar-degeneration-due-to-a-paraneoplastic-phenomenon-associated-with-metastatic-merkel-cell-carcinoma-a-case-report
#16
Angelos Sharobeam, Jason Ray, Juliana Dong, Victor Chong
PURPOSE: The aim of this article is to illustrate the diagnostic challenges and management of paraneoplastic neurological syndromes in Merkel cell carcinoma. MATERIALS AND METHODS: We describe a previously functionally independent 85-year-old woman who presented with subacute onset of dizziness and gait ataxia in the setting of metastatic Merkel cell carcinoma. RESULTS: Diagnosis was made on biopsy after positron emission tomography imaging revealed increased metabolic activity in 2 left inguinofemoral lymph nodes...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28836741/quantification-and-neurochemical-coding-of-the-myenteric-plexus-in-humans-no-regional-variation-between-the-distal-colon-and-rectum
#17
K-S Ng, N A Montes-Adrian, D A Mahns, M A Gladman
BACKGROUND: It remains unclear whether regional variation exists in the human enteric nervous system (ENS) ie, whether intrinsic innervation varies along the gut. Recent classification of gastrointestinal neuropathies has highlighted inadequacies in the quantification of the human ENS. This study used paired wholemounts to accurately quantify and neurochemically code the hindgut myenteric plexus, comparing human distal colon and rectum. METHODS: Paired human descending colonic/rectal specimens were procured from 15 patients undergoing anterior resection...
March 2018: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/28767032/subacute-supranuclear-palsy-in-anti-hu-paraneoplastic-encephalitis
#18
Masaki Ohyagi, Satoru Ishibashi, Takuya Ohkubo, Zen Kobayashi, Hidehiro Mizusawa, Takanori Yokota, Hirofumi Emoto, Motohiro Kiyosawa
No abstract text is available yet for this article.
July 2017: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/28682959/paraneoplastic-neuropathies
#19
REVIEW
Jean-Christophe Antoine, Jean-Philippe Camdessanché
PURPOSE OF REVIEW: To review recent advances in paraneoplastic neuropathies with emphasis on their definition, different forms and therapeutic development. RECENT FINDINGS: A strict definition of definite paraneoplastic neuropathies is necessary to avoid confusion. With carcinoma, seronegative sensory neuronopathies and neuronopathies and anti-Hu and anti-CV2/Contactin Response Mediator Protein 5 antibodies are the most frequent. With lymphomas, most neuropathies occur with monoclonal gammopathy including AL amyloidosis, Polyneuropathy-Organomegaly-Endocrinopathy-M component-Skin changes (POEMS) syndrome, type I cryoglobulinemia and antimyelin-associated glycoprotein (MAG) neuropathies and Waldenström's disease...
October 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28588791/synchronous-adie-s-syndrome-and-type-1-antineuronal-nuclear-antibody-anti-hu-related-paraneoplastic-neurological-syndromes-as-predictors-of-complete-response-in-limited-stage-small-cell-lung-cancer-a-case-report
#20
Katherina Bernadette Sreter, Blazenka Barisic, Marija Barisic Kutija, Suzana Kukulj, Miroslav Samarzija
Adie's syndrome (AS) and paraneoplastic sensorimotor neuropathy with cerebellar ataxia (PSN CA) are extremely rare, rapidly progressive, autoimmune diseases associated with the development of antibodies against neuronal-specific Hu proteins that are abnormally expressed in small-cell lung cancer (SCLC). We herein present the unique case of a 55-year-old obese woman, previous heavy smoker, who, during treatment with standard cisplatin-etoposide chemotherapy for limited-stage SCLC, developed simultaneous AS and worsening symptoms consistent with PSN CA that led to significant neurological disability and severe axonal electrophysiological pattern on nerve conduction studies...
June 2017: Molecular and Clinical Oncology
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