Database : MEDLINE
Search on : Paraneoplastic and Syndromes [Words]
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[PMID]: 29512373
[Au] Autor:Tavakol M; Mahdaviani SA; Ghaemi MR; Vaezi M; Dorudinia A; Jamaati H; Velayati AA
[Ad] Address:Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran AND Department of Allergy and Clinical Immunology, Shahid Bahonar Hospital, Alborz University of Medical Sciences, Karaj, Iran.
[Ti] Title:Good's Syndrome-Association of the Late Onset Combined Immunodeficiency with Thymoma: Review of Literature and Case Report.
[So] Source:Iran J Allergy Asthma Immunol;17(1):85-93, 2018 Feb.
[Is] ISSN:1735-1502
[Cp] Country of publication:Iran
[La] Language:eng
[Ab] Abstract:Good's syndrome, the adult onset hypogammaglobulinemia associated with thymoma has been explained about six decades ago. It generally presents with recurrent infections and several paraneoplastic syndromes including myasthenia gravis, pure red cell aplasia, connective tissue disorders, superior vena cava, Horner's syndrome, lichen planus and inflammatory bowel disease. Lack of B cell, dysfunction of T cell, CD4+ T cell lymphopenia, reversed CD4/CD8+ T cell ratio, autoantibodies against Th17 related cytokines have been respected as the pathogenesis of the immune dysregulation this syndrome. A 57-year-old man was admitted to our hospital with a history of thymectomy due to thymoma (Type A) 6 years ago. He developed weight loss and recurrent persistent diarrhea caused by isospora belli. His chest CT scan revealed bilateral bronchiectasis. His laboratory data showed hypogammaglobulinemia and he was treated by monthly IVIG with the diagnosis of good's syndrome. Nevertheless he referred again with left sided loss of vision because of CMV retinitis and he also developed nail candidiasis. Good's syndrome should be considered in every patient with a history of thymoma and recurrent infection. Immunologic evaluation of these patients including measurement of the serum level of immunoglobulin as well as B cell and T cell subgroups should be performed. Physicians must be aware and think about this entity in patients with adult onset immunodeficiency.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[St] Status:In-Data-Review

  2 / 10494 MEDLINE  
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[PMID]: 29489644
[Au] Autor:Attademo L; De Falco S; Rosanova M; Esposito M; Mazio F; Foschini F; Santaniello A; Fiore G; Matano E; Manganelli F; Carlomagno C
[Ad] Address:Department of Clinical Medicine and Surgery.
[Ti] Title:A case report of limbic encephalitis in a metastatic colon cancer patient during first-line bevacizumab-combined chemotherapy.
[So] Source:Medicine (Baltimore);97(9):e0011, 2018 Mar.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:RATIONALE: Paraneoplastic limbic encephalitis (PLE) is one of the most common causes of neurologic paraneoplastic syndromes, with unclear pathogenesis. While several reports published in the last decades showed the occurrence of PLE in a variety of cancers, only a few cases have been associated with colon cancer. PATIENT CONCERNS: In February 2017, a 54-year-old man with clinical history of radically resected colon cancer started first line chemotherapy with FOLFOXIRI plus bevacizumab, after radiological diagnosis of multiple liver and bone metastases. During the third cycle of treatment, the patient developed psychomotor agitation and hallucinations followed by severe consciousness level reduction and cognitive impairment. DIAGNOSES: Magnetic resonance imaging showed hyperintense signals in both hippocampal areas, insula and right cingulate gyrus on fluid attenuated inversion recovery, diffusion weighted imaging, and T2-weighted images, highly suggestive of limbic encephalitis. Other causes (brain metastases, toxicity of chemotherapeutic agents, and infections) were excluded. INTERVENTIONS: Empirical immunosuppressive treatment (high-dose immunoglobulins and corticosteroids) was administered and chemotherapy was resumed. OUTCOMES: A slowly progressive improvement in neurological condition has been observed, even though radiological signs of limbic encephalitis are still evident. LESSONS: The present case highlights the complex diagnostic process of PLE, and the lack of a standard treatment. Moreover, the absence of correlation between PLE and tumor progression or tumor burden, and the opportunity of treating underlying neoplasm is discussed.
[Mh] MeSH terms primary: Antineoplastic Agents, Immunological/therapeutic use
Antineoplastic Combined Chemotherapy Protocols/therapeutic use
Bevacizumab/therapeutic use
Colonic Neoplasms/drug therapy
Colonic Neoplasms/pathology
Limbic Encephalitis/diagnosis
[Mh] MeSH terms secundary: Bone Neoplasms/drug therapy
Bone Neoplasms/secondary
Colonic Neoplasms/complications
Electroencephalography
Humans
Limbic Encephalitis/complications
Liver Neoplasms/drug therapy
Liver Neoplasms/secondary
Magnetic Resonance Imaging
Male
Middle Aged
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Antineoplastic Agents, Immunological); 2S9ZZM9Q9V (Bevacizumab)
[Em] Entry month:1803
[Cu] Class update date: 180305
[Lr] Last revision date:180305
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:180301
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000010011

  3 / 10494 MEDLINE  
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[PMID]: 29395322
[Au] Autor:Hor JY; Lim TT; Cheng MC; Chia YK; Wong CK; Lim SM; Cheah CF; Tan K; Easaw PES; Leite MI
[Ad] Address:Department of Neurology, Penang General Hospital, Penang, Malaysia; Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom. Electronic address: horjy@yahoo.com.
[Ti] Title:Thymoma-associated myasthenia gravis and LGI1-encephalitis, with nephrotic syndrome post-thymectomy.
[So] Source:J Neuroimmunol;317:100-102, 2018 Apr 15.
[Is] ISSN:1872-8421
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Thymoma is associated with a wide spectrum of autoimmune paraneoplastic syndromes, though it is uncommon for multiple paraneoplastic syndromes to be present in a single individual. We report a rare case of an elderly gentleman who was found to have thymoma-associated myasthenia gravis and LGI1-encephalitis with myokymia, who presented with nephrotic syndrome (minimal change glomerulopathy) after thymectomy. The latter two paraneoplastic syndromes had manifested when prednisolone was tapered down to low dose. This case serves to remind neurologists that apart from paraneoplastic neurological manifestations, thymoma may also be associated with renal disease. Nephropathy in myasthenia patients with thymoma should be properly evaluated, as it is treatable with immunotherapy, and it may even occur post-thymectomy.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180304
[Lr] Last revision date:180304
[St] Status:In-Data-Review

  4 / 10494 MEDLINE  
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[PMID]: 29494437
[Au] Autor:Kim AE; Kang P; Bucelli RC; Ferguson CJ; Schmidt RE; Varadhachary AS; Day GS
[Ad] Address:Departments of Neurology.
[Ti] Title:Autoimmune Encephalitis With Multiple Autoantibodies: A Diagnostic and Therapeutic Challenge.
[So] Source:Neurologist;23(2):55-59, 2018 Mar.
[Is] ISSN:2331-2637
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:INTRODUCTION: Indications for autoantibody testing in patients with rapid-onset cognitive impairment have expanded in step with the growing number of disease-associated autoantibodies and clinical syndromes. Although increased access to autoantibody testing has broadened our understanding of the spectrum of autoimmune encephalitis (AE), it has also produced new challenges associated with deciphering the contributions of disease-associated autoantibodies in patients with atypical clinical features and/or multiple autoantibodies. These challenges are illustrated through presentation of a patient with AE associated with autoantibodies against intracellular and cell-surface neuronal antigens. The implications of detection of multiple autoantibodies are considered in the context of relevant literature, and used to frame a diagnostic and therapeutic approach. CASE REPORT: A previously well 67-year-old man presented with encephalopathy and psychosis, impaired visual fixation, and ataxia, emerging over 3 months. Hu, CRMP-5, and NMDAR autoantibodies were identified in the cerebrospinal fluid. No malignancy was discovered despite extensive investigations. An aggressive course of immunotherapy temporarily stabilized his course; however, the patient succumbed to his illness 10 months after symptom onset. Lack of sustained response to immunotherapy and neuropathologic findings suggested that AE associated with Hu antibodies was primarily responsible for this patient's progressive decline. CONCLUSIONS: Multiple autoantibodies may be detected in patients with AE. When antibodies targeting intracellular and cell-surface antigens are detected together, investigation and treatment of syndromes associated with intracellular antibodies should be prioritized, acknowledging the link between these antibodies and irreversible neuronal injury. In paraneoplastic cases, prognosis may be tied to early detection and treatment of the underlying malignancy.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180301
[Lr] Last revision date:180301
[St] Status:In-Process
[do] DOI:10.1097/NRL.0000000000000171

  5 / 10494 MEDLINE  
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[PMID]: 29198644
[Au] Autor:Fuente García C; González-López JJ; Muñoz-Negrete FJ; Rebolleda G
[Ad] Address:Servicio de Oftalmología, Hospital Universitario Ramón y Cajal, Instituto Ramón y Cajal de Investigaciones Sanitarias (IRYCIS), Madrid, España.
[Ti] Title:La utilidad diagnóstica del electrorretinograma negativo. The diagnostic usefulness of the negative electroretinogram.
[So] Source:Arch Soc Esp Oftalmol;93(3):126-135, 2018 Mar.
[Is] ISSN:1989-7286
[Cp] Country of publication:Spain
[La] Language:eng; spa
[Ab] Abstract:The definition of the negative response of the full field electroretinogram is the presence of a b-wave with less amplitude than the a-wave (b/a ratio<1) in the combined response of cones and rods. The presence of this pattern reflects an alteration in the bipolar cells, the Müller cells, or in the transmission of the stimulus from the photoreceptors to the bipolar cells, with preserved photoreceptor function. This finding can be seen bilaterally and symmetrically in different hereditary conditions, such as congenital stationary night blindness, juvenile X-linked retinoschisis, and Duchenne and Becker muscular dystrophies. On the other hand, it can also be found unilaterally (or asymmetrically) in acquired pathologies, such as some types of immuno-mediated retinitis (Birdshot retinochoroiditis), autoimmune retinopathies, cancer/melanoma associated retinopathy, or retinal toxicity. The objective of this review is to summarise the characteristics of the pathologies in which this finding can be observed, in order to highlight its usefulness in the differential diagnosis of retinal conditions.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180227
[Lr] Last revision date:180227
[St] Status:In-Process

  6 / 10494 MEDLINE  
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[PMID]: 29284772
[Au] Autor:Patial T; Sharma K; Thakur D; Gupta G
[Ad] Address:Department of General Surgery, Indira Gandhi Medical College, Shimla, Himachal Pradesh 171001, India.
[Ti] Title:Consumptive hypothyroidism: an unusual paraneoplastic manifestation of a gastric gastrointestinal stromal tumor.
[So] Source:Exp Oncol;39(4):319-321, 2017 Dec.
[Is] ISSN:1812-9269
[Cp] Country of publication:Ukraine
[La] Language:eng
[Ab] Abstract:A 42-year-old hypothyroid shepherd presented with a progressive abdominal lump accompanied by nausea and abdominal fullness. In addition, he had worsening hypothyroidism, despite supranormal doses of thyroxine. Computed tomography of the abdomen was suggestive of a mass lesion in relation to the stomach. A resection of the mass was done and the histopathology was suggestive of gastrointestinal stromal tumor. After surgery, the patient became euthyroid. We believe the patient had consumptive hypothyroidism due to the tumor.
[Mh] MeSH terms primary: Gastrointestinal Stromal Tumors/complications
Gastrointestinal Stromal Tumors/pathology
Hypothyroidism/etiology
Paraneoplastic Syndromes/etiology
[Mh] MeSH terms secundary: Adult
Humans
Hypothyroidism/pathology
Male
Paraneoplastic Syndromes/pathology
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180223
[Lr] Last revision date:180223
[Js] Journal subset:IM
[Da] Date of entry for processing:171230
[St] Status:MEDLINE

  7 / 10494 MEDLINE  
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[PMID]: 29443727
[Au] Autor:Lyu S; Zhang M; Gao Y
[Ad] Address:Department of Ophthalmology, West China Hospital of Sichuan University, Wuhou, Chengdu, Sichuan, China.
[Ti] Title:Acute bilateral retina hemorrhages beneath internal limiting membrane: An unusual ophthalmological case report of acute leukemia during complete clinical remission.
[So] Source:Medicine (Baltimore);97(7):e0000, 2018 Feb.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:RATIONALE: Leukemia is a common hematologic disease that causes various systemic complications, such as ophthalmological disorders. The venous congestion is considered to be the main clinical sign that occurs during the initial stage of the disease, whereas white-centered hemorrhages are the most typical manifestations in leukemic retinopathy. These complications usually manifest when the disease presents with clinical and hematological symptoms. In the present study, we report a patient who was diagnosed with leukemic retinopathy. The initial signs of this disease were bilateral hemorrhages that occurred during complete clinical remission. Previous studies regarding this condition are quite rare. PATIENT CONCERNS: We report a 26-year-old man who was diagnosed with leukemic retinopathy and exhibited the initial signs of the disease, namely bilateral hemorrhages with a distinct appearance beneath the internal limiting membrane. In addition, flame-shaped hemorrhages were observed surrounding the optic discs and/or along the vessels in the absence of venous congestion. All these changes were present during complete clinical remission. DIAGNOSES: Bilateral acute leukemic retinopathy, acute lymphoblastic leukemia (pro-B lymphocyte, BCR-ABL chimeric gene-positive). INTERVENTIONS: Clinical remission was achieved following effective systemic chemotherapy that was applied for leukemia in the hematology department. A dynamic observation was applied actively in the absence of surgery and/or medical treatment for ophthalmologic treatment. OUTCOMES: Best corrected visual acuity was 20/40 in the right eye and 20/60 in the left eye, which was considerably better than those noted at the initial visit of the patient (20/250 in the right eye and 20/400 in the left eye). LESSONS: The cautious expectant treatment is safe and helpful for acute leukemic retinopathy. A long-term follow-up is inevitable. Effective systemic chemotherapy that is required for leukemia treatment can achieve clinical remission, which might be helpful in controlling the pathological changes of the eyes.
[Mh] MeSH terms primary: Paraneoplastic Syndromes, Ocular/complications
Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications
Retinal Hemorrhage/etiology
[Mh] MeSH terms secundary: Acute Disease
Adult
Humans
Male
Retina/pathology
Retinal Hemorrhage/pathology
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180222
[Lr] Last revision date:180222
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:180215
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000010000

  8 / 10494 MEDLINE  
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[PMID]: 29191778
[Au] Autor:Padda SK; Yao X; Antonicelli A; Riess JW; Shang Y; Shrager JB; Korst R; Detterbeck F; Huang J; Burt BM; Wakelee HA; Badve SS
[Ad] Address:Department of Medicine, Division of Oncology, Stanford Cancer Institute/Stanford University School of Medicine, Stanford, California. Electronic address: padda@stanford.edu.
[Ti] Title:Paraneoplastic Syndromes and Thymic Malignancies: An Examination of the International Thymic Malignancy Interest Group Retrospective Database.
[So] Source:J Thorac Oncol;13(3):436-446, 2018 Mar.
[Is] ISSN:1556-1380
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:INTRODUCTION: Thymic epithelial tumors (TETs) are associated with paraneoplastic/autoimmune (PN/AI) syndromes. Myasthenia gravis is the most common PN/AI syndrome associated with TETs. METHODS: The International Thymic Malignancy Interest Group retrospective database was examined to determine (1) baseline and treatment characteristics associated with PN/AI syndromes and (2) the prognostic role of PN/AI syndromes for patients with TETs. The competing risks model was used to estimate cumulative incidence of recurrence (CIR) and the Kaplan-Meier method was used to calculate overall survival (OS). A Cox proportional hazards model was used for multivariate analysis. RESULTS: A total of 6670 patients with known PN/AI syndrome status from 1951 to 2012 were identified. PN/AI syndromes were associated with younger age, female sex, thymoma histologic type, earlier stage, and an increased rate of total thymectomy and complete resection status. There was a statistically significant lower CIR in the group with a PN/AI syndrome than in the group without a PN/AI syndrome (10-year CIR 17.3% versus 21.2%, respectively [p = 0.0003]). The OS was improved in the group with a PN/AI syndrome compared to the group without a PN/AI syndrome (median OS 21.6 years versus 17.0 years, respectively [hazard ratio = 0.63, 95% confidence interval: 0.54-0.74, p < 0.0001]). However, in the multivariate model for recurrence-free survival and OS, PN/AI syndrome was not an independent prognostic factor. DISCUSSION: Previously, there have been mixed data regarding the prognostic role of PN/AI syndromes for patients with TETs. Here, using the largest data set in the world for TETs, PN/AI syndromes were associated with favorable features (i.e., earlier stage and complete resection status) but were not an independent prognostic factor for patients with TETs.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180223
[Lr] Last revision date:180223
[St] Status:In-Data-Review

  9 / 10494 MEDLINE  
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[PMID]: 29464446
[Au] Autor:Jin XF; Spampatti MP; Spitzweg C; Auernhammer CJ
[Ad] Address:Department of Internal Medicine IV, University-Hospital Campus Grosshadern, Ludwig-Maximilian University of Munich, Munich, Germany.
[Ti] Title:Supportive therapy in gastroenteropancreatic neuroendocrine tumors: Often forgotten but important.
[So] Source:Rev Endocr Metab Disord;, 2018 Feb 20.
[Is] ISSN:1573-2606
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:Neuroendocrine tumors (NETs) are a group of rare and heterogeneous malignancies that can develop in various organs. A significant number of gastroenteropancreatic neuroendocrine tumours (GEP-NETs) is functionally active and presents with symptoms related to the secretion of biologically active substances, leading to the development of distinct clinical syndromes. There are various therapeutic approaches for GEP-NETs, including curative surgery, palliative surgery, local-ablative and loco-regional therapies as well as systemic therapeutic options including peptide receptor radionuclide therapy, cytotoxic therapy, and molecularly targeted therapies. Specific supportive therapy of patients with NETs includes management or prevention of hormone-related clinical syndromes and paraneoplastic states. Supportive therapy plays a key role in NET treatment. Supportive therapy includes debulking surgery and interventional radiologic techniques to reduce tumour bulk or load, as well as systemic medical treatment options to manage or prevent hypersecretion syndromes and treatment-related side effects. Supportive therapies are a type of of comprehensive treatment addressing the patient as a whole person throughout the process of NET treatment. Therefore, supportive therapy also encompasses psychosocial support, expert nursing, nutritional support and management of cancer related pain.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1802
[Cu] Class update date: 180221
[Lr] Last revision date:180221
[St] Status:Publisher
[do] DOI:10.1007/s11154-018-9443-6

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[PMID]: 29313654
[Au] Autor:Latimer KM
[Ad] Address:US Naval Hospital Sigonella Italy, PSC 836 Box 2670, FPO, AE 09636.
[Ti] Title:Lung Cancer: Clinical Presentation and Diagnosis.
[So] Source:FP Essent;464:23-26, 2018 Jan.
[Is] ISSN:2159-3000
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:In the absence of screening, most patients with lung cancer are not diagnosed until later stages, when the prognosis is poor. The most common symptoms are cough and dyspnea, but the most specific symptom is hemoptysis. Digital clubbing, though rare, is highly predictive of lung cancer. Symptoms can be caused by the local tumor, intrathoracic spread, distant metastases, or paraneoplastic syndromes. Clinicians should suspect lung cancer in symptomatic patients with risk factors. The initial study should be chest x-ray, but if results are negative and suspicion remains, the clinician should obtain a computed tomography scan with contrast. The diagnostic evaluation for suspected lung cancer includes tissue diagnosis, staging, and determination of functional capacity, which are completed simultaneously. Tissue samples should be obtained using the least invasive method possible. Management is based on the individual tumor histology, molecular testing results, staging, and performance status. The management plan is determined by a multidisciplinary team consisting of a pulmonology subspecialist, medical oncology subspecialist, radiation oncology subspecialist, and thoracic surgeon. The family physician should remain involved with the patient to ensure that patient priorities are supported and, if necessary, to arrange for end-of-life care.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180220
[Lr] Last revision date:180220
[St] Status:In-Process


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