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https://www.readbyqxmd.com/read/28920766/clinical-profile-of-patients-with-paraneoplastic-neuromyelitis-optica-spectrum-disorder-and-aquaporin-4-antibodies
#1
Maria Sepúlveda, Nuria Sola-Valls, Domingo Escudero, Bojan Rojc, Manuel Barón, Luis Hernández-Echebarría, Begoña Gómez, Josep Dalmau, Albert Saiz, Francesc Graus
BACKGROUND: In a minority of patients with neuromyelitis optica spectrum disorder (NMOSD) and aquaporin-4 antibodies (AQP4-IgG), the disease has a paraneoplastic origin. It is unknown whether these patients have distinctive clinical features. OBJECTIVE: To report the clinical features of a series of patients with paraneoplastic NMOSD and AQP4-IgG and to review previously reported cases. METHODS: Retrospective analysis of clinical records of 156 patients with NMOSD and AQP4-IgG and review of previously reported patients with paraneoplastic NMOSD and AQP4-IgG...
September 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28913957/autologous-peripheral-blood-stem-cell-transplantation-and-the-role-of-lenalidomide-in-patients-affected-by-poems-syndrome
#2
REVIEW
Francesco Autore, Idanna Innocenti, Marco Luigetti, Nicola Piccirillo, Federica Sora, Patrizia Chiusolo, Simona Sica, Andrea Bacigalupo, Luca Laurenti
POEMS syndrome is a rare paraneoplastic condition, with a poorly understood pathogenesis. High dose chemotherapy followed by autologous stem cell transplantation (ASCT) has been reported to be an effective therapeutic option for patients with good performance status. Here, we review the role of ASCT for POEMS syndrome and discuss indications together with advantages and disadvantages, and related issues such lenalidomide given before or after ASCT, VEGF levels as a marker of disease, and different regimens for stem cell mobilization...
September 15, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28912913/paraneoplastic-dermatomyositis-syndrome-presenting-as-dysphagia
#3
Emmanuel Ofori, Daryl Ramai, Mel Ona, Madhavi Reddy
Dermatomyositis (DM) is a rare autoimmune condition which predominantly affects females in the fifth and sixth decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with a heliotropic rash around the eyes, and Gottron's papules on extensor surfaces of the hands. While the pathophysiology of DM is still unclear, abnormal T- and B-cell immune activity has been reported. DM has been associated with malignancy and has been shown to appear before, concurrently with, or after diagnosis of cancer...
August 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28906499/-incomplete-poems-syndrome-with-multicentric-castleman-s-disease
#4
P González de la Aleja, M García-Navarro, R Sánchez-Rodríguez, J M Ramos-Rincón
Castleman's disease (CD) is an atypical lymphoproliferative disorder of unknown cause, characterized by non-clonal nodal hyperplastic growth. Two forms of clinical presentation are currently recognized, one localized and the other multicentric, and four histopathologic variants. It is characterized by generalized lymphadenopathy, hepatosplenomegaly, fever and night sweats. CD may present severe pancytopenia, multi-organ failure, lymphoma evolution and it can sometimes be associated with paraneoplastic syndromes such as POEMS syndrome...
September 14, 2017: Anales del Sistema Sanitario de Navarra
https://www.readbyqxmd.com/read/28905135/a-case-of-reversible-anti-nmda-receptor-encephalitis-neuropsychological-and-neuroradiological-features
#5
Carlotta Mutti, Federica Barocco, Lucia Zinno, Anna Negrotti, Marco Spallazzi, Giovanni Pavesi, Simona Gardini, Paolo Caffarra
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune encephalitis mainly affecting young women. We report a case of a mild paraneoplastic anti-NMDAR encephalitis in a 31-year-old female with an ovarian immature teratoma. The patient exhibited a severe short-term episodic memory impairment and psychiatric symptoms. A detailed diagnostic work-up including complete clinical and laboratory examinations, neuropsychological assessments, and neuroradiological investigations has been done at the onset and during follow-up...
September 13, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28904989/antibodies-to-trim46-are-associated-with-paraneoplastic-neurological-syndromes
#6
Marleen H van Coevorden-Hameete, Sam F B van Beuningen, Matthieu Perrenoud, Lena M Will, Esther Hulsenboom, Jean-Francois Demonet, Lidia Sabater, Johan M Kros, Jan J G M Verschuuren, Maarten J Titulaer, Esther de Graaff, Peter A E Sillevis Smitt, Casper C Hoogenraad
Paraneoplastic neurological syndromes (PNS) are often characterized by the presence of antineuronal antibodies in patient serum or cerebrospinal fluid. The detection of antineuronal antibodies has proven to be a useful tool in PNS diagnosis and the search for an underlying tumor. Here, we describe three patients with autoantibodies to several epitopes of the axon initial segment protein tripartite motif 46 (TRIM46). We show that anti-TRIM46 antibodies are easy to detect in routine immunohistochemistry screening and can be confirmed by western blotting and cell-based assay...
September 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28904568/pediatric-autoimmune-encephalitis
#7
REVIEW
Massimo Barbagallo, Giovanna Vitaliti, Piero Pavone, Catia Romano, Riccardo Lubrano, Raffaele Falsaperla
Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28900071/a-case-of-non-islet-cell-tumor-hypoglycemia-nicth-associated-with-gastrointestinal-stromal-tumor-gist
#8
John M Wilson, Jessica Ginsberg, Karen Cutts, Steve Urban
BACKGROUND Non-islet cell tumor hypoglycemia (NICTH) is a newly recognized, but uncommon, paraneoplastic syndrome that is associated with tumors of mesenchymal origin. We report a case of NICTH associated with a gastrointestinal stromal tumor (GIST). CASE REPORT A 60-year-old man presented to the emergency department of our hospital after being found unconscious in his home. His serum blood glucose on hospital admission was 40 mg/dL. He reported a three-month history of diffuse abdominal pain, fatigue, and blurred vision...
September 13, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28895465/paraneoplastic-cushing-syndrome-case-series-and-review-of-the-literature
#9
Annelies Deldycke, Christel Haenebalcke, Youri Taes
OBJECTIVES: Paraneoplastic Cushing syndrome is a rare condition, representing a small fraction of the adrenocorticotropic hormone (ACTH)-dependent cases of Cushing syndrome Methods: Four case descriptions and literature review, highlighting the diagnostic challenges and treatment options are presented. RESULTS: Different tumor types can be associated with ectopic ACTH secretion. The most common types are bronchial carcinoids and small cell lung carcinoma (SCLC)...
September 12, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28885355/secondary-hypertrophic-osteoarthropathy-caused-by-non-pleural-or-pulmonary-tumors
#10
MULTICENTER STUDY
Hans-Jonas Meyer, Leonard Leifels, Andreas Gunter Bach, Alexey Surov
Hypertrophic osteoarthropathy (HOA) is a rare paraneoplastic syndrome characterized by digital clubbing, periosteal reaction, polyarthralgia, arthritis, and synovitis. Herein, we report a case series of patients with secondary HOA caused by non-pleural or pulmonary tumors.The radiologic databases of 2 tertiary university hospitals were retrospectively screened for secondary HOA patients. In addition, a systemic review of the published case reports. Only HOA cases with non-pleural or pulmonary malignancies were involved into the study...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28885235/corticosteroids-therapy-for-paraneoplastic-nephrotic-syndrome
#11
Rukma Parthvi, Sunil Mehra, Musa Njeru
No abstract text is available yet for this article.
September 2017: American Journal of Therapeutics
https://www.readbyqxmd.com/read/28884051/anti-n-methyl-d-aspartate-receptor-nmdar-encephalitis-in-association-with-ovarian-teratoma
#12
Javaad Ahmad, Muhammad Saad Sohail, Amina Khan, Ahmed H Qavi, Pramod Gaudel, Mehr Zahid, Salman Assad
Anti-N-methyl-D-aspartate-Receptor (NMDAR) encephalitis is an autoimmune disorder with a multifaceted presentation that involves memory deficits, psychiatric symptoms, and autonomic instability. This case report describes the classic presentation of Anti-NMDAR encephalitis and highlights its association with ovarian teratomas. We present a 26 -year-old female who came in with new onset seizures and altered mentation who subsequently developed automatism. Electroencephalograms (EEG) showed left frontal spikes and right temporal delta activity...
July 5, 2017: Curēus
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
#13
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...
September 6, 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
#14
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/28877826/clinical-analysis-of-unicentric-castleman-s-disease-with-paraneoplastic-pemphigus-and-bronchiolitis-obliterans
#15
Jun-Feng Zhen, Lu Zhang, Xin-Xin Cao, Jun Feng, Dao-Bin Zhou, Song-Bai Lin, Jian Li
Objective To investigate the clinical features of unicentric Castleman's disease(UCD)with paraneoplastic pemphigus(PNP)and bronchiolitis obliterans(BO).Method Data of UCD patients with PNP and BO from Peking Union Medical College Hospital were retrospectively analyzed,along with literatures review. Results Totally 23 cases(11 males and 12 females)were enrolled.The median age was 31 years(13-56 years).The most common pathological type was hyaline-vascular variant(91.4%),and most tumors located in abdominopelvic cavity(69...
August 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28877745/erythrocytosis-caused-by-giant-chromophobe-renal-cell-carcinoma-a-case-report-indicating-a-9-year-misdiagnosis-of-polycythemia-vera
#16
Renbo Guo, Yiran Liang, Lei Yan, Zhonghua Xu, Juchao Ren
BACKGROUND: Erythrocytosis, a rare paraneoplastic syndrome, generally occurs in patients with clear cell renal cell carcinoma and has never been reported in patients with chromophobe renal cell carcinoma. CASE PRESENTATION: We report a case of a young man suffering from a giant (22-cm) mass on his left kidney. Because of a history of polycythemia vera, the patient had been treated for the condition for 9 years. Radical nephrectomy was successfully performed, and the postoperative pathologic examination confirmed a diagnosis of chromophobe renal cell carcinoma...
September 6, 2017: Chinese Journal of Cancer
https://www.readbyqxmd.com/read/28875953/roles-of-loss-of-chromosome-14q-allele-in-the-prognosis-of-renal-cell-carcinoma-with-c-reactive-protein-abnormity
#17
Gang Wang, Da-Ming Zhang, Hai-Ying Zhuang, Chao Yin, Jing Liu, Zi-Chun Wang, Li-Cheng Cai, Ming-Hua Ren, Wan-Hai Xu, Cheng Zhang
Background: Renal cell carcinoma (RCC) is frequently associated with paraneoplastic inflammatory syndrome (PIS). This study aimed at exploring the connections between the survival rate and specific gene alterations and the potential mechanism. Methods: We retrospectively studied 69 surgical RCC cases from August 2014 to February 2016, including 18 cases of clear cell RCC (ccRCC) demonstrating elevated pretreatment serum C-reactive protein (CRP, Group A). Twelve of the 18 cases were symptomized with febrile episode...
September 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28871260/dermatomyositis-and-immune-mediated-necrotizing-myopathies-a-window-on-autoimmunity-and-cancer
#18
REVIEW
Audrey Aussy, Olivier Boyer, Nadège Cordel
Autoimmune myopathies (myositides) are strongly associated with malignancy. The link between myositis and cancer, originally noticed by Bohan and Peter in their classification in 1975 (1), has been evidenced by large population-based cohort studies and a recent meta-analysis. The numerous reports of cases in which the clinical course of myositis reflects that of cancer and the short delay between myositis and cancer onset support the notion that myositis may be an authentic paraneoplastic disorder. Thus, cancer-associated myositis raises the question of cancer as a cause rather than a consequence of autoimmunity...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28860940/limbic-encephalitis-a%C3%A2-report-of-four-cases
#19
Żanna Pastuszak, Adam Stępień, Kazimierz Tomczykiewicz, Renata Piusińska-Macoch, Joanna Kordowska, Dariusz Galbarczyk, Jarosław Świstak
Usually limbic encephalitis (LE) is a paraneoplastic neurologic syndrome. LE symptoms can precede cancer even by a few years. Almost 50% of LE cases are connected with small cell lung carcinoma. Testis and breast cancers, granulomatous disease, thymoma, and teratomas are also often connected with LE. Other cases have infectious and autoimmunological aetiology. In LE limbic system dysfunction is observed, and it is accompanied by cerebellum and brain stem abnormalities as well as polyneuropathy. Paraneoplastic limbic encephalitis is sometimes a part of larger syndrome in which brain stem and spinal cord are involved in an inflammatory process called paraneoplastic encephalomyelitis...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/28860867/doege-potter-syndrome-cause-of-nonislet-cell-tumor-hypoglycemia-the-first-case-report-from-nepal
#20
Vivek Pant, Suman Baral, Gita Sayami, Prakash Sayami
Doege-Potter syndrome (DPS), a paraneoplastic syndrome, presents as a hypoinsulinemic hypoglycemia from the ectopic secretion of insulin-like growth factor II from a solitary fibrous tumor which may be intrapleural or extrapleural in origin. We report a case of severe hypoglycemia in a 70-year old female initially admitted for resection of left sided solitary fibrous tumor of pleura. Investigation revealed true hypoglycemia, and DPS was diagnosed. The tumor was completely resected, after which no further hypoglycemic episodes were seen in 2 years follow-up...
2017: International Medical Case Reports Journal
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