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neurologic paraneoplastic

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https://www.readbyqxmd.com/read/29784613/cryoglobulinemic-glomerulonephritis-associated-with-nodal-and-renal-infiltration-by-t-cell-lymphoma-of-t-follicular-helper-phenotype-a-case-report
#1
Jennifer Li, Marille Umakanathan, Chow Heok P'ng, Winny Varikatt, Fiona Kwok, Ming-Wei Lin, Mirna Vucak-Dzumhur
We present a unique case of cryoglobulinemic glomerulonephritis associated with nodal and renal infiltration by T-cell lymphoma of T-follicular helper phenotype. The patient presented with transient neurologic symptoms, severe nephritic syndrome with nephrotic-range proteinuria, and acute kidney injury. He had elevated double-stranded DNA levels, low complement levels, detectable cryoglobulin, and detectable immunoglobulin M (IgM) paraprotein. The kidney biopsy showed cryoglobulinemic glomerulonephritis with a membranoproliferative pattern and diffuse interstitial infiltrates on light microscopy; IgM, C3 but weak IgG, C1q, and negative C4d staining on immunofluorescence; and deposits with organized substructures on electron microscopy...
May 18, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29784239/paraneoplastic-syndromes-in-ophthalmology
#2
REVIEW
A Bussat, S Langner-Lemercier, A Salmon, F Mouriaux
Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. They are unrelated to local tumor invasion or metastasis and unexplained by nutritional, metabolic, infectious or iatrogenic causes. The presence of antibodies may aid in the diagnosis of a paraneoplastic syndrome, although this is not an absolute requirement. Chorioretinal involvement is seen in CAR (cancer-associated retinopathy) syndrome, MAR (melanoma-associated retinopathy) syndrome, paraneoplastic vitelliform maculopathy, and bilateral diffuse uveal melanocytic proliferation...
May 18, 2018: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29781194/paraneoplastic-autoimmune-encephalitis-associated-with-pleomorphic-lung-carcinoma-an-autopsy-case-report
#3
Takashi Ando, Yoji Goto, Kazuo Mano, Fumio Nomura, Masako Kurashige, Masafumi Ito, Maya Mimuro, Yasushi Iwasaki, Masahisa Katsuno, Mari Yoshida
A 64-year-old man was admitted with acute onset disturbed consciousness. Cerebrospinal fluid analysis revealed pleocytosis and elevated protein, with negative cultures and PCR. Serum antibodies for autoimmune encephalitis were also negative. Brain magnetic resonance imaging (MRI) was unremarkable, but whole-body CT scan showed a tumor in the left lower lung lobe. Bronchial brush cytology demonstrated clusters of malignant cells, and 18 F-fluorodeoxyglucose positron emission tomography showed multiple lesions and increased uptake in the lung tumor...
May 20, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29766473/mr-imaging-findings-in-some-rare-neurological-complications-of-paediatric-cancer
#4
REVIEW
Tetsuhiko Okabe, Taiki Nozaki, Noriko Aida, Jay Starkey, Mikako Enokizono, Tetsu Niwa, Atsuhiko Handa, Yuji Numaguchi, Yasuyuki Kurihara
Neurological complications of paediatric cancers are a substantial problem. Complications can be primary from central nervous system (CNS) spread or secondary from indirect or remote effects of cancer, as well as cancer treatments such as chemotherapy and radiation therapy. In this review, we present the clinical and imaging findings of rare but important neurological complications in paediatric patients with cancer. Neurological complications are classified into three phases: pre-treatment, treatment and post-remission...
May 15, 2018: Insights Into Imaging
https://www.readbyqxmd.com/read/29761119/antibodies-against-cell-adhesion-molecules-and-neural-structures-in-paraneoplastic-neuropathies
#5
Ana M Siles, Eugenia Martínez-Hernández, Josefa Araque, Jordi Diaz-Manera, Ricard Rojas-Garcia, Eduard Gallardo, Isabel Illa, Francesc Graus, Luis Querol
Objective: Paraneoplastic neurological syndromes (PNS) are rare neurological disorders in which ectopic expression of neural antigens by a tumor results in an autoimmune attack against the nervous system. Onconeural antibodies not only guide PNS diagnosis but may also help detecting underlying malignancies. Our project aims to uncover new potential antibodies in paraneoplastic neuropathies (PN). Methods: Thirty-four patients fulfilling diagnostic criteria of possible ( n = 9; 26...
May 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29742813/mutated-cancer-autoantigen-implicated-cause-of-paraneoplastic-myasthenia-gravis
#6
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/29742721/coexistence-of-lambert-eaton-myasthenic-syndrome-and-autoimmune-encephalitis-with-anti-crmp5-cv2-and-anti-gabab-receptor-antibodies-in-small-cell-lung-cancer-a-case-report
#7
Hongfang Li, Aimei Zhang, Yanlei Hao, Hongzhi Guan, Zhanyun Lv
RATIONALE: Autoimmune encephalitis and Lambert-Eaton myasthenic syndrome are classic paraneoplastic neurological conditions common in patients with small cell lung cancer. PATIENT CONCERNS: The patient complained of tiredness, fluctuating recent memory loss, and inability to find his home. His family members reported a change in character, irritability, and paranoia. One month later, the patient had 1 grand mal seizure lasting 5 minutes. DIAGNOSIS: The patient was diagnosed with limbic encephalitis combined with Lambert-Eaton myasthenic syndrome...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29727049/multifactorial-analysis-of-opsoclonus-myoclonus-syndrome-etiology-tumor-vs-no-tumor-in-a-cohort-of-356-us-children
#8
Michael R Pranzatelli, Elizabeth D Tate, Nathan R McGee
BACKGROUND: Pediatric opsoclonus-myoclonus syndrome (OMS) presents a paradox of etiopathogenesis: A neuroblastic tumor (NB) is found in only one half of the cases, the others are ascribed to infections or designated as idiopathic. METHOD: From an IRB-approved observational study of 356 US children with OMS, secondary analysis of "etiology" and related factors was performed on a well-characterized cohort. The "Tumor" (n = 173) and "No Tumor" groups (n = 183), as defined radiologically, were compared according to multiple factors considered potentially differentiating...
May 4, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29725842/motor-neuron-disease-of-paraneoplastic-origin-a-rare-but-treatable-condition
#9
Nicolas Mélé, Giulia Berzero, Thierry Maisonobe, François Salachas, Guillaume Nicolas, Nicolas Weiss, Guillemette Beaudonnet, Francois Ducray, Dimitri Psimaras, Timothée Lenglet
Paraneoplastic motor neuron disorders (MND) are rare conditions; their exact clinical and electrophysiological phenotype have not been exhaustively described yet. The purpose of this study is to depict the main characteristics of paraneoplastic MND to highlight the features that may allow its diagnosis. Based on the description of eight original cases, and on the revision of 21 patients identified from a systematic review of the literature, the main features of paraneoplastic MND can be summarized as follows: (1) subacute; (2) lower motor neuron syndrome, associated or not with upper motor neuron involvement; (3) predominant asymmetric upper limb involvement; (4) presence of other non-motor neurological manifestations, including sensory neuronopathy; (5) signs of inflammation in the cerebrospinal fluid (CSF); (6) neurological improvement or stabilization after immunotherapy and tumor treatment...
May 3, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29720343/-neurogenic-autonomic-dysfunction-in-adults
#10
Astrid Juhl Terkelsen, John Hansen, Anja Klostergaard, Marit Otto, Henning Mølgaard, Christian Lodberg Hvas, Klaus Krogh, Hans Jørgen Kirkeby, Henning Andersen, Troels Staehelin Jensen
Neurogenic autonomic dysfunction (NAD) is underdiagnosed, and it is likely in patients, who have orthostatic hypotension and symptoms from multiple organ systems as well as abnormal results from a neurological examination. A clinical and neurophysiological examination of the autonomic nervous system combined with a standardised paraclinical evaluation should be performed. NAD may be present in neurodegenerative disorders, vitamin deficiency, toxicity, infection, and in paraneoplastic, metabolic, hereditary and immune-mediated conditions...
April 30, 2018: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/29718880/various-clinical-features-of-patients-with-anti-hu-associated-paraneoplastic-neurological-syndromes-an-observational-study
#11
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
#12
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/29681644/paraneoplastic-lambert-eaton-syndrome-in-a-patient-with-disseminated-metastatic-cancer
#13
Gregorio Arellano-Aguilar, Erik Santiago Núñez-Mojica, José Luis Gutiérrez-Velazco, Luis Gerardo Domínguez-Carrillo
Background: Neurological paraneoplastic syndromes are rare, occur in 0.01% of all cancer patients; like part of them, the Lambert-Eaton syndrome is an autoimmune presynaptic disorder of neuromuscular transmission characterized by muscle weakness and neurovegetative dysfunction, and often associated with small cell lung cancer. Case report: A 72 years old female with a family history of lung cancer and leukemia, with 7 months of dry cough and 3 months with waist and pelvic muscle weakness, oropharyngeal dysphagia, dry mouth, chronic constipation and weight loss of 10 kg...
2018: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/29644085/protein-molecular-modeling-shows-residue-t599-is-critical-to-wild-type-function-of-polg-and-description-of-a-novel-variant-associated-with-the-sando-phenotype
#14
John E Richter, Hector G Robles, Elizabeth Mauricio, Ahmed Mohammad, Paldeep S Atwal, Thomas R Caulfield
Sensory ataxic neuropathy with dysarthria and ophthalmoparesis (SANDO) is a rare phenotype resulting from pathogenic variants of mitochondrial DNA polymerase gamma ( POLG ). We modeled a novel POLG variant, T599P, that causes the SANDO phenotype and another variant at the same residue, p.T599E, to observe their effect on protein function and confirm the pathogenicity of T599P. Through neoteric molecular modeling techniques, we show that changes at the T599 residue position introduce extra rigidity into the surrounding helix-loop-helix, which places steric pressure on nearby nucleotides...
2018: Human Genome Variation
https://www.readbyqxmd.com/read/29613897/immune-mediated-myelopathies
#15
Dean M Wingerchuk
PURPOSE OF REVIEW: This article reviews the clinical presentation, diagnostic evaluation, and management of immune-mediated myelopathies. RECENT FINDINGS: The discovery of several neural autoantibodies and their antigenic targets has revolutionized the investigation and treatment of immune-mediated myelopathies. Detection of these serologic biomarkers can support or establish a diagnosis of an autoimmune myelopathy, and, in the case of paraneoplastic syndromes, indicate the likely presence of an underlying malignancy...
April 2018: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29613896/neoplastic-myelopathies
#16
Jing Wu, Surabhi Ranjan
PURPOSE OF REVIEW: This article discusses the diagnosis and management of neoplasms that affect the spinal cord as well as spinal cord disorders that can occur due to cancer treatments. RECENT FINDINGS: Neoplastic myelopathies are uncommon neurologic disorders but cause significant morbidity when they occur. Primary spinal cord tumors can be classified into intramedullary, intradural extramedullary, or extradural tumors. Diffuse gliomas and ependymal tumors are the most common intramedullary tumors...
April 2018: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29545765/clinical-characteristics-and-etiology-of-bilateral-vestibular-loss-in-a-cohort-from-central-illinois
#17
Jorge C Kattah
Background: Previous series of bilateral vestibular loss (BVL) identified numerous etiologies, but surprisingly, a cause in a significant number of cases remains unknown. In an effort to understand possible etiology and management strategies, a global effort is currently in progress. Here, I contribute my 10-year experience with both acute and chronic BVL during the 2007-2017 decade. Methods: This is a retrospective review of the charts and EMR of patients diagnosed with BVL in the last 10 years...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29535092/ovarian-teratoma-associated-with-anti-nmda-n-methyl-d-aspartate-receptor-encephalitis
#18
Nellihela Leel, Hemanshoo S Thakkar, David Drake, Nordeen Bouhadiba
Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a paraneoplastic, immune-mediated encephalopathy with a known association with ovarian teratomas. We present the first case in the UK of a 7-year-old patient presenting with this condition. Having been previously fit and healthy, the patient presented with an acute onset of cognitive disturbances. The initial suspicion was that of an infective encephalopathy; however, the lumbar puncture results were negative. Electroencephalography reported diffuse background slowing, supporting a diagnosis of encephalopathy...
March 13, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29526952/lambert-eaton-myasthenic-syndrome-associated-with-synchronous-double-cancer-a-combination-of-small-cell-carcinoma-of-the-cervix-and-breast-carcinoma
#19
Hiroyuki Fukuda, Akira Tanaka, Yasuyuki Hirashima, Ichiro Ito
Lambert-Eaton myasthenic syndrome (LEMS) is most commonly associated with small cell lung carcinoma, while it is rarely associated with gynecological and breast carcinoma. We herein report a case of LEMS associated with synchronous double cancer, which was a combination of small cell carcinoma of the cervix and breast carcinoma. The early diagnosis and treatment of LEMS are important for achieving a good outcome. The possibility of accompanying paraneoplastic neurological syndrome must be sufficiently considered in gynecology and breast cancer patients...
March 9, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29518508/ilio-iliac-arteriovenous-fistulae-an-unusual-diagnosis-with-an-even-more-unusual-clinical-presentation
#20
Andreia Coelho, Pedro Brandão, Miguel Lobo, Ignatio Lojo, Alexandra Canedo
BACKGROUND: Major pelvic ilio-iliac arteriovenous fistula (AVF) is an exceedingly rare diagnosis with only a few described cases in the literature, most of them related to congenital defects or trauma. In this case report, we aim to present a case of an ilio-iliac AVF with an atypical clinical presentation. METHODS: Relevant medical data were collected from hospital database. RESULTS: The patient is a 77-year-old woman, with a relevant medical history of a temporally remote hysterectomy...
March 5, 2018: Annals of Vascular Surgery
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