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    Evolution, immunity and the emergence of brain superautoantigens.
    F1000Res 2017 21;6:171. Epub 2017 Feb 21.
    Bank of Tissues and Cells, Lyon University Hospital (Hospices Civils de Lyon), CarMeN Laboratory, INSERM 1060, INRA 1397, INSA Lyon, Université Claude Bernard Lyon-1, Lyon, F-69000, France.
    While some autoimmune disorders remain extremely rare, others largely predominate the epidemiology of human autoimmunity. Notably, these include psoriasis, diabetes, vitiligo, thyroiditis, rheumatoid arthritis and multiple sclerosis. Thus, despite the quasi-infinite number of "self" antigens that could theoretically trigger autoimmune responses, only a limited set of antigens, referred here as superautoantigens, induce pathogenic adaptive responses. Read More

    [Small cell lung cancer associated with multiple paraneoplastic syndromes].
    Biomedica 2017 Jan 24;37(1):8-10. Epub 2017 Jan 24.
    Department of Internal Medicine, Mayo Clinic AZ, Scottsdale, United States of America.
    We report the case of a patient presenting with multiple severe electrolyte disturbances who was subsequently found to have small cell lung cancer. Upon further evaluation, she demonstrated three distinct paraneoplastic processes, including the syndrome of inappropriate antidiuretic hormone, Fanconi syndrome, and an inappropriate elevation in fibroblast growth factor-23 (FGF23). The patient underwent one round of chemotherapy, but she was found to have progressive disease. Read More

    Subacute Noninfective Inflammatory Encephalopathy: Our Experience and Diagnostic Problems.
    Indian J Psychol Med 2017 Mar-Apr;39(2):183-187
    Department of Neuromicrobiology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
    Introduction: Immune dysregulation associated encephalopathies present with significant psychiatric manifestations and only a few soft neurological and general systemic features. They are generally resistant to treatment with psychiatric medications. Generalized orthostatic myoclonus and faciobrachial dystonic seizures are mistaken as Creutzfeldt-Jakob disease and subacute sclerosing panencephalitis. Read More

    Autoimmune Voltage-Gated Potassium Channelopathy Presenting With Catecholamine Excess.
    Pediatr Neurol 2017 Apr 8. Epub 2017 Apr 8.
    Department of Pediatrics, Maine Medical Center's Barbara Bush Children's Hospital, Portland, Maine.
    Background: Autoimmune voltage-gated potassium channelopathies have been associated with a range of neurological presenting symptoms, including central, peripheral, and autonomic dysfunction.

    Patient Description: We describe a 12-year-old boy who presented with nine months of pain, anxiety, and 30-pound weight loss. He was admitted for failure to thrive, then noted to be persistently hypertensive and tachycardic. Read More

    Video-assisted extirpation of cranial mediastinal masses in dogs: 18 cases (2009-2014).
    J Am Vet Med Assoc 2017 Jun;250(11):1283-1290
    OBJECTIVE To characterize clinical findings, surgical procedures, complications, and outcomes in dogs undergoing extirpation of masses from the cranial mediastinum via video-assisted thoracic surgery (VATS) and establish preliminary guidelines for case selection when considering VATS for thymectomy in dogs. DESIGN Retrospective case series. ANIMALS 18 client-owned dogs that underwent extirpation of a cranial mediastinal mass by means of VATS at 5 academic referral hospitals from 2009 through 2014. Read More

    Demographic characteristics, aetiology, and assessment of treatment options in leukocytoclastic vasculitis.
    Postepy Dermatol Alergol 2017 Apr 13;34(2):104-109. Epub 2017 Apr 13.
    Department of Dermatovenereology, Medical Faculty, Bezmialem Vakif University, Istanbul, Turkey.
    Introduction: Vasculitides are a heterogeneous group of diseases characterized by inflammation of the blood vessel walls. Etiological factors include infections, drugs, connective tissue diseases, and malignancies.

    Aim: To examine the demographic characteristics, etiological factors, and treatment options in 75 patients with leukocytoclastic vasculitis. Read More

    Anti-N-Methyl-d-Aspartate Receptor Encephalitis Appearing as a New-Onset Psychosis: Disease Course in Children and Adolescents Within the California Encephalitis Project.
    Pediatr Neurol 2017 Feb 4. Epub 2017 Feb 4.
    Department of Psychiatry, UCSF-Fresno, Fresno, California.
    Background: Given that psychiatric symptoms are common, not only during the course of the illness but also on presentation, in children and adolescents with anti-N-methyl-d-aspartate receptor encephalitis, it is important that practitioners in the field of child and adolescent psychiatry possess an adequate understanding of the clinical features and potential treatment of this disease. We describe the clinical characteristics of 24 patients who presented to the California Encephalitis Project.

    Methods: Patients were referred by physicians, and standardized forms were used to gather demographic, clinical, and laboratory data. Read More

    Impact of Autoantibodies against Glycolytic Enzymes on Pathogenicity of Autoimmune Retinopathy and Other Autoimmune Disorders.
    Front Immunol 2017 28;8:505. Epub 2017 Apr 28.
    School of Medicine, Casey Eye Institute, Oregon Health and Science University, Portland, OR, USA.
    Autoantibodies (AAbs) against glycolytic enzymes: aldolase, α-enolase, glyceraldehyde-3-phosphate dehydrogenase, and pyruvate kinase are prevalent in sera of patients with blinding retinal diseases, such as paraneoplastic [cancer-associated retinopathy (CAR)] and non-paraneoplastic autoimmune retinopathies, as well as in many other autoimmune diseases. CAR is a degenerative disease of the retina characterized by sudden vision loss in patients with cancer and serum anti-retinal AAbs. In this review, we discuss the widespread serum presence of anti-glycolytic enzyme AAbs and their significance in autoimmune diseases. Read More

    Paraneoplastic acral vascular syndrome in a patient with metastatic melanoma under immune checkpoint blockade.
    BMC Cancer 2017 May 12;17(1):327. Epub 2017 May 12.
    Department of Dermatology, Ruhr-University Bochum, Gudrunstr. 56, 44791, Bochum, Germany.
    Background: Paraneoplastic acral vascular syndrome (PAVS) is a rare phenomenon which is observed in patients with adenocarcinomas and other malignancies. Various potential pathogenic mechanisms such as tumour invasion of sympathetic nerves, hyperviscosity, hypercoagulability, vasoactive tumour-secreted substances, and immunological mechanisms have been suggested.

    Case Presentation: We report a 60-year-old Caucasian male attended our hospital with a bulky lymph node mass in the right axilla. Read More

    Pemphigus.
    Nat Rev Dis Primers 2017 May 11;3:17026. Epub 2017 May 11.
    Department of Dermatology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.
    Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. Read More

    Diagnosing neuroleukemiosis: Is there a role for (18)F-FDG-PET/CT?
    Rev Esp Med Nucl Imagen Mol 2017 May 4. Epub 2017 May 4.
    PET Unit, Department of Nuclear Medicine - IDI, Hospital Universitari de Bellvitge - IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain.
    An imaging case is presented on a patient referred to our department for an (18)F-FDG-PET/CT, as a paraneoplastic syndrome was suspected due to his clinical situation. He had a history of acute myeloid leukemia (AML) treated two years earlier, with sustained complete remission to date. (18)F-FDG-PET/CT findings revealed hypermetabolism in almost all nerve roots, suggesting meningeal spread, consistent with the subsequent MRI findings. Read More

    Juvenile dermatomyositis/polymyositis and lymphoma.
    J Neurol Sci 2017 Jun 27;377:19-24. Epub 2017 Mar 27.
    Department of Neurology, Weill Cornell Medicine/New York Presbyterian Hospital, 525 East 68th Street, New York, NY 10065-4885, USA. Electronic address:
    In patients with juvenile dermatomyositis/polymyositis (JDM/PM), malignancy is a rare phenomenon. An extensive workup for neoplastic disease is not routinely indicated, but should be considered if unusual physical findings are noted at JDM/PM diagnosis or during follow-up period. The objective of this literature review was to assess for any association between, and disease profile of, JDM/PM and lymphoma in childhood. Read More

    Atypical thymic carcinoid manifesting with nephrotic-range proteinuria in a 7-year-old boy.
    Pediatr Nephrol 2017 May 5. Epub 2017 May 5.
    Department of Pediatric Cardiology, Nephrology and Hypertension, Poznan University of Medical Sciences, 27/33 Szpitalna Str, 60-572, Poznan, Poland.
    Background: Nephrotic-range proteinuria as a paraneoplastic syndrome (PNS) is an exceptional presentation, especially in children. It is usually associated with hematologic malignancies. Solid tumors are very rare causes of proteinuria. Read More

    Managing Renal Cell Carcinoma Associated Paraneoplastic Syndrome with Nephron-sparing Surgery in a Patient with von Hippel-Lindau.
    Urol Case Rep 2017 Jul 27;13:101-103. Epub 2017 Apr 27.
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bldg. 10, Hatfield Clinical Research Center, Rm 2 W-3952 10 Center Drive MSC 1210, 20892-1210, Bethesda, MD, USA.
    A patient with germline von Hippel-Lindau (VHL) gene alteration and history of multiple tumors present with classical paraneoplastic syndrome (PNS) associated with renal cell carcinoma (RCC). She underwent open nephron sparing surgery with resolution of symptoms. She remained without recurrence of RCC for the initial 2 years of her follow-up. Read More

    A Case of Sweet's Syndrome Secondary to Myelodysplastic Syndrome - Diagnostic and Treatment Challenges.
    Maedica (Buchar) 2016 Jun;11(2):154-157
    Department of Onco-hematology, "Maria Sklodowska Curie" Children's Emergency Hospital, Bucharest, Romania.
    Sweet's Syndrome also knows as acute febrile neutrophilic dermatosis, is a rare skin's condition, that can occur either idiopathic or secondary. In the case of the latter, the syndrome can develop after certain malignancies (paraneoplastic syndrome), because of exposure to some medication or post infectious. It is more frequent in women aged between 30 and 50 years, but concerning children, the disorder is extremely rare (8% of the total number of cases), having equal sex ratio distribution. Read More

    Octreotide is Ineffective in Treating Tumor-Induced Osteomalacia; Results of a Short-Term Therapy.
    J Bone Miner Res 2017 Apr 29. Epub 2017 Apr 29.
    Skeletal Clinical Studies Unit, Craniofacial and Skeletal Disease Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD, USA.
    Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome in which unregulated hypersecretion of fibroblast growth factor 23 (FGF23) by phosphaturic mesenchymal tumors (PMT) causes renal phosphate wasting, hypophosphatemia, and osteomalacia. The resulting mineral homeostasis abnormalities and skeletal manifestations can be reversed with surgical resection of the tumor. Unfortunately, PMTs are often difficult to locate, and medical treatment with oral phosphate and vitamin D analogues is either insufficient to manage the disease or not tolerated. Read More

    Cochlear Impairment and Autoimmune Ear Disorder in a Patient with Breast Cancer.
    Audiol Res 2017 Feb 13;7(1):165. Epub 2017 Mar 13.
    Tinnitus Center, European Hospital, Rome.
    The purpose of this study was to consider the possible role of autoimmune diseases and paraneoplastic syndrome in the genesis of tinnitus. The incidence of autoimmune inner ear disease (AIED) is rare, accounting for <1% of all cases of hearing impairment and dizziness. In presence of auditory and vestibular deficit in oncological patients, a paraneoplastic syndrome with cochleovestibulopathy should be considered. Read More

    [Polyneuropathy in the elderly].
    Z Gerontol Geriatr 2017 Apr 28. Epub 2017 Apr 28.
    Universitätsklinik für Geriatrie, Christian-Doppler-Klinik, Universitätsklinikum Salzburg, Paracelsus Medizinische Privatuniversität, Ignaz-Harrer-Str. 79, 5020, Salzburg, Österreich.
    The peripheral nervous system is subject to changes during the ageing process, e. g. deep tendon reflexes decrease, as does proprioception. Read More

    βhCG-secreting osteosarcoma.
    BMJ Case Rep 2017 Apr 28;2017. Epub 2017 Apr 28.
    Mater Misericordiae University Hospital, Dublin, Ireland.
    A 35-year-old woman presented to our institution with tender, right knee swelling. Radiological and pathological work-up revealed metastatic osteosarcoma. Prior to the initiation of chemotherapy, she underwent a routine urine pregnancy test and was surprised with a positive result. Read More

    Motor neuropathies and lower motor neuron syndromes.
    Rev Neurol (Paris) 2017 May 20;173(5):320-325. Epub 2017 Apr 20.
    Reference Centre for Neuromuscular Diseases and ALS, University Hospital La Timone, 264 rue Saint Pierre, 13385 Marseille cedex 05, France. Electronic address:
    Motor or motor-predominant neuropathies may arise from disease processes affecting the motor axon and/or its surrounding myelin. Lower motor neuron syndrome (LMNS) arises from a disease process affecting the spinal motor neuron itself. The term LMNS is more generally used, rather than motor neuronopathy, although both entities are clinically similar. Read More

    The role of tumor board conferences in neuro-oncology: a nationwide provider survey.
    J Neurooncol 2017 Apr 18. Epub 2017 Apr 18.
    Department of Neurosurgery, Henry Ford Health System, 2799 W Grand Blvd, Detroit, MI, 48202, USA.
    The tumor board or multidisciplinary cancer meeting (MCM) is the foundation of high value multidisciplinary oncology care, coordinating teams of specialists. Little is known on how these meetings are implemented in Neuro-oncology. Benefits of MCMs include coordination, direction for complicated cases, education, and a forum for communication, emerging technology, and clinical trials. Read More

    Biotherapy in Inflammatory Diseases of the CNS: Current Knowledge and Applications.
    Curr Treat Options Neurol 2017 May;19(5):19
    Département de Neurologie, CHU de Strasbourg, Strasbourg, France.
    Opinion Statement: Biotherapy represents an innovative therapeutic approach that includes immunotherapy (vaccines, apheresis, and antibodies); gene therapy; and stem cell transplants. Their development helps to cross the bridge from bench to bedside and brings new hope of a cure for severe diseases in different fields of medicine. In neurology, a growing range of applications is being developed for these medications. Read More

    Paraneoplastic limbic encephalitis in a patient with extensive disease small-cell lung cancer.
    Mol Clin Oncol 2017 Apr 10;6(4):575-578. Epub 2017 Feb 10.
    Department of Oncology, Jagiellonian University Medical College, 31-531 Krakow, Poland.
    Paraneoplastic limbic encephalitis (PLE) is a rare disorder infrequently accompanying malignancy, coexisting in ~50% of the cases with small-cell lung cancer (SCLC). The pathomechanism of PLE is considered to be immune-mediated, with production of specific anti-Hu antibodies and activation of T-cells directed against onconeural antigens present on both tumor cells and neurons. We herein report the case of a 50-year-old male patient who, prior to being diagnosed with SCLC, presented with typical symptoms of PLE (seizures, subacute cognitive dysfunction with severe memory impairment, anxiety and hallucinations). Read More

    Cancer is a propagandist.
    Stud Hist Philos Biol Biomed Sci 2017 Jun 11;63:28-31. Epub 2017 Apr 11.
    Department of Kinesiology and Nutrition, University of Illinois at Chicago, Chicago, IL, USA. Electronic address:
    Communication among cells (also known as cross-talk) plays a prominent role in the current knowledge of the pathophysiology of cancer and of cancer-associated conditions such as paraneoplastic syndromes and cachexia that are responsible for much of cancer's morbidity and mortality. Yet, biomedical scientists lack an explicit unifying frame that places this exchange of molecular information at the core of their understanding of cancer as a systemic disease. Propaganda is a type of information that aims at misleading, a form of communication intended primarily to serve the messenger. Read More

    An Unfortunate Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Change (POEMS).
    Cureus 2017 Mar 8;9(3):e1086. Epub 2017 Mar 8.
    Internal Medicine, University of Central Florida College of Medicine.
    POEMS syndrome is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes, which is a rare paraneoplastic disease of monoclonal plasma cells. A mandatory criterion to diagnose POEMS syndrome is the presence of a monoclonal plasma cell dyscrasia in which plasma cell leukemia is the most aggressive form. Early identification of the features of the POEMS syndrome is critical for patients to identify an underlying plasma cell dyscrasias and to reduce the morbidity and mortality of the disease by providing early therapy. Read More

    Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease?
    Hematol Oncol 2017 Apr 11. Epub 2017 Apr 11.
    Department of Haematology, University Hospitals of Bristol NHS Trust, Bristol, UK.
    Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. Read More

    Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy.
    Ann Dermatol 2017 Apr 24;29(2):219-222. Epub 2017 Mar 24.
    Department of Dermatology, Gangnam Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
    Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Read More

    Adaptive Immunity Is the Key to the Understanding of Autoimmune and Paraneoplastic Inflammatory Central Nervous System Disorders.
    Front Immunol 2017 23;8:336. Epub 2017 Mar 23.
    Department of Neurology, Neuroimmunology, University of Regensburg , Regensburg , Germany.
    There are common aspects and mechanisms between different types of autoimmune diseases such as multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSDs), and autoimmune encephalitis (AE) as well as paraneoplastic inflammatory disorders of the central nervous system. To our present knowledge, depending on the disease, T and B cells as well as antibodies contribute to various aspects of the pathogenesis. Possibly the events leading to the breaking of tolerance between the different diseases are of great similarity and so far, only partially understood. Read More

    Paraneoplastic Glomerulopathy in a Case of Collecting Duct Renal Cell Carcinoma.
    J Clin Diagn Res 2017 Feb 1;11(2):OD03-OD04. Epub 2017 Feb 1.
    Professor and Head, Department of Nephrology, Kasturba Hospital, Manipal University , Manipal, Karnataka, India .
    Paraneoplastic glomerulopathy has been described in established cases of the solid tumors of lung, gastrointestinal system, breast, etc., and rarely in patients with Renal Cell Carcinoma (RCC). Studies on secondary glomerular diseases have described a higher incidence of IgA nephropathy in patients with RCC compared to membranous glomerulopathy, which are commonly reported in malignancies of the lung and gastrointestinal tract. Read More

    Inflammatory Myopathies with Cutaneous Involvement: from Diagnosis to Therapy.
    Folia Med (Plovdiv) 2017 Mar;59(1):7-13
    Department of Dermatology and Venereology, Medical University of Sofia, 1 St Georgi Sofiiski St., 1431 Sofia.
    The group of idiopathic inflammatory myopathies (IIM) include various disorders of skeletal muscles with or without skin involvement. The most common types are dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). Dermatomyositis subdivides into various clinical forms such as juvenile, amyopathic or paraneoplastic dermatomyositis, scleromyositis, overlap or anti-synthetase syndromes, etc. Read More

    Pure Red Cell Aplasia Associated with Good Syndrome.
    Korean J Thorac Cardiovasc Surg 2017 Apr 5;50(2):119-122. Epub 2017 Apr 5.
    Department of Thoracic Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital.
    Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature. Read More

    Idiopathic SIADH in young patients: don't forget the nose.
    Acta Otorhinolaryngol Ital 2017 Feb;37(1):76-79
    Institute of Radiology, Catholic University of Sacred Heart, Rome, Italy.
    Olfactory neuroblastoma (ONB) is an uncommon neuroendocrine sinonasal cancer associated by many authors to ectopic production of several biologically active substances. We report a case of a 31-year-old male patient who presented with idiopathic syndrome of inappropriate secretion of antidiuretic hormone (SIADH). During diagnostic work-up, a CT scan of the head was performed and an ethmoidal ONB was detected. Read More

    Retinitis Pigmentosa and Other Dystrophies.
    Dev Ophthalmol 2017 28;58:191-201. Epub 2017 Mar 28.
    Retinitis pigmentosa (RP) is a heterogeneous group of inherited retinal degenerations characterized by progressive degeneration of rod and cone cells that affects predominantly peripheral visual fields. Macular edema may cause additional central visual acuity decrease. Cystoid macular edema (CME) is one of the few treatable causes of visual loss in RP. Read More

    CD4+ and CD8+ T cells are both needed to induce paraneoplastic neurological disease in a mouse model.
    Oncoimmunology 2017 9;6(2):e1260212. Epub 2016 Dec 9.
    INSERM UMR1043 - CNRS U5282, Centre de Physiopathologie Toulouse-Purpan, Toulouse, France; Université Toulouse III, Tolouse, France.
    Paraneoplastic neurological disorders (PNDs) are rare human autoimmune diseases that mostly affect the central nervous system (CNS). They are triggered by an efficient immune response against a neural self-antigen that is ectopically expressed in neoplastic tumors. Due to this shared antigenic expression, the immune system reacts not only to tumor cells but also to neural cells resulting in neurological damage. Read More

    Ectopic ACTH-producing large cell neuroendocrine Pancoast tumour presenting as Horner syndrome.
    BMJ Case Rep 2017 Mar 24;2017. Epub 2017 Mar 24.
    Department of Family Medicine, Apple Valley Medical Clinic, Apple Valley, Minnesota, USA.
    We present an interesting case where a patient is presented with a droopy left eyelid (as part of Horner syndrome) and Cushingoid features which were a result of a Pancoast tumour (apical lung tumour in superior pulmonary sulcus) involving the left lung. This tumour was secreting ectopic adrenocorticotropic hormone (ACTH), a paraneoplastic endocrine phenomenon, which resulted in Cushing syndrome symptomatology. Though most ectopic ACTH-producing lung cancers are either small cell or carcinoid tumours, this was in fact a large cell neuroendocrine cancer (LCNEC). Read More

    Comparison of surgical approach and extent of resection for Masaoka-Koga Stage I and II thymic tumours in Europe, North America and Asia: an International Thymic Malignancy Interest Group retrospective database analysis†.
    Eur J Cardiothorac Surg 2017 Feb 28. Epub 2017 Feb 28.
    Division of Thoracic Surgery, Swedish Cancer Institute, Seattle, WA, USA.
    Objectives: Surgeons at different institutions worldwide choose different types of operations for thymic tumours. It is not known whether these differences affect the outcomes of the patients.

    Methods: A total of 1430 patients with Masaoka-Koga pathological Stage I-II thymic tumours without myasthenia gravis or pre-treatment were identified from the International Thymic Malignancy Interest Group retrospective database. Read More

    Sweet Syndrome.
    J Cutan Med Surg 2017 Feb 1:1203475417690719. Epub 2017 Feb 1.
    1 Department of Dermatology, Hospital das Clínicas, University of São Paulo Medical School, Sao Paulo, SP, Brazil.
    Background: Sweet syndrome (SS) is an infrequent skin disease characterised by sudden onset of fever, leukocytosis, neutrophilia, and tender erythematous plaques infiltrated by neutrophils. Multiple conditions have been associated with this syndrome.

    Objectives: The aim of this study was to evaluate the clinical, epidemiological, laboratory, and histopathological findings and associations of patients with SS. Read More

    POEMS Syndrome: an Enigma.
    Curr Hematol Malig Rep 2017 Apr;12(2):85-95
    Division of Hematology, Medicine and Oncology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
    POEMS syndrome is a paraneoplastic disorder secondary to an underlying plasma cell dyscrasia. By definition, all patients with POEMS syndrome must display polyneuropathy and monoclonal plasma cell disorder. In addition, at least one major criterion (Castleman's disease, sclerotic bone lesions, or vascular endothelial growth factor elevation) and one minor criterion (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia) are required for diagnosis. Read More

    Etiological profile of noncompressive myelopathies in a tertiary care hospital of Northeast India.
    Ann Indian Acad Neurol 2017 Jan-Mar;20(1):41-50
    Department of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India.
    Background: The discovery of antibodies against aquaporin-4 and evolving concepts of noncompressive myelopathies in the 21(st) century have made a major impact on the etiological profile of these diseases, with few cases turning out to be idiopathic.

    Objective: To find causes of noncompressive myelopathy in a tertiary care hospital of Northeast India.

    Materials And Methods: An observational study was carried out in the Neurology Department of Gauhati Medical College, Guwahati, from September 2013 to February 2016. Read More

    Autoimmune paraneoplastic syndromes associated to lung cancer: A systematic review of the literature: Part 2: Hematologic, cutaneous and vascular syndromes.
    Lung Cancer 2017 Apr 30;106:93-101. Epub 2017 Jan 30.
    Service des Soins Intensifs et Urgences Oncologiques & Thoracic Oncology, Institut Jules Bordet, Centre des Tumeurs de l'Université Libre de Bruxelles (ULB), Belgium; Laboratoire Facultaire de Médecine Factuelle (ULB), Belgium. Electronic address:
    The development of new immune treatment in oncology and particularly for lung cancer may induce new complications, particularly activation or reactivation of auto-immune diseases. In this context, a systematic review on the auto-immune paraneoplastic syndromes associated with lung cancer appears useful. This article is the second of a series of five and deals with hematologic, cutaneous and vascular syndromes. Read More

    Autoimmune paraneoplastic syndromes associated to lung cancer: A systematic review of the literature: Part 3: Neurological paraneoplastic syndromes, involving the central nervous system.
    Lung Cancer 2017 Apr 30;106:83-92. Epub 2017 Jan 30.
    Service des Soins Intensifs et Urgences Oncologiques & Thoracic Oncology, Institut Jules Bordet, Centre des Tumeurs de l'Université Libre de Bruxelles (ULB), Belgium; Laboratoire facultaire de Médecine factuelle (ULB), Belgium. Electronic address:
    The development of new immune treatment in oncology and particularly for lung cancer may induce new complications, particularly activation or reactivation of auto-immune diseases. In this context, a systematic review on the auto-immune paraneoplastic syndromes that can complicate lung cancer appears useful. This article is the third of a series of five and deals mainly with neurological paraneoplastic syndromes involving the central nervous system. Read More

    Autoimmune paraneoplastic syndromes associated to lung cancer: A systematic review of the literature.
    Lung Cancer 2017 Apr 30;106:102-109. Epub 2017 Jan 30.
    Service des Soins Intensifs et Urgences Oncologiques & Oncologie thoracique, Institut Jules Bordet, Centre des Tumeurs de l'Université Libre de Bruxelles (ULB) et, Laboratoire Facultaire de Médecine Factuelle (ULB), Belgium. Electronic address:
    The development of new immune treatment in oncology and particularly for lung cancer may induce new complications, particularly activation or reactivation of auto-immune diseases. In this context, a systematic review on the auto-immune paraneoplastic syndromes associated with lung cancer appears useful. This article is the first of a series of five and deals with the methodology applied for the review and with renal and rheumatic syndromes. Read More

    The effect of myasthenia gravis as a prognostic factor in thymoma treatment.
    North Clin Istanb 2016 25;3(3):194-200. Epub 2017 Jan 25.
    Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
    Objective: Thymoma is a standard epithelial tumor. Though it is rare, it constitutes 50% of anterior mediastinal masses. Variety of immunological diseases may accompany thymoma; however, myasthenia gravis (MG) is the most frequently associated paraneoplastic syndrome. Read More

    Acrokeratosis paraneoplastica (Bazex syndrome) - a systematic review on risk factors, diagnosis, prognosis and management.
    J Eur Acad Dermatol Venereol 2017 Mar 8. Epub 2017 Mar 8.
    Klinik für Hautkrankheiten, Universitätsklinikum, Jena, Deutschland.
    Acrokeratosis paraneoplastica Bazex (Bazex syndrome) is a rare paraneoplastic skin disease defined by erythematous, violaceous, scaly plaques on the hands and feet and on other acral locations such as nose and ears. Bazex syndrome is linked to a variety of underlying malignancies. Usually the skin lesions develop prior to the diagnosis of an internal malignant neoplasm with spontaneous remission after tumour removal. Read More

    Anti-N-methyl-D-aspartate receptor encephalitis associated with hepatic neuroendocrine carcinoma: A case report.
    J Clin Neurosci 2017 Mar 2. Epub 2017 Mar 2.
    National Neuroscience Institute, Singapore General Hospital Campus, 20 College Road, Academia 169856, Singapore. Electronic address:
    Anti-N-methyl-D-aspartate receptor (Anti-NMDAR) encephalitis can present with and without tumor. Tumor associations are less common in older patients. We report a 65-year-old gentleman who presented with one week history of cough, chills, rigor and altered behavior, followed by florid visual and auditory hallucinations. Read More

    Diagnostic Value of Positron Emission Tomography Combined with Computed Tomography for Evaluating Critically Ill Neurological Patients.
    Front Neurol 2017 14;8:33. Epub 2017 Feb 14.
    Department of Intensive Care, University Medical Center Hamburg-Eppendorf , Hamburg , Germany.
    Purpose: (18)F-fluorodeoxyglucose positron emission tomography combined with computed tomography (FDG-PET/CT) is a promising new tool for the identification of inflammatory, infectious, and neoplastic foci. The aim of our work was to evaluate the diagnostic value of FDG-PET/CT in patients treated on a neurological/neurosurgical ICU or stroke unit.

    Methods: We performed a single-center, 10-year, retrospective evaluation of the value of FDG-PET/CT in critically ill adult patients with severe neurological disease. Read More

    [Review of the psychiatric aspects of anti-NMDA (N-methyl-D-aspartic acid) receptor encephalitis, case report, and our plans for a future study].
    Neuropsychopharmacol Hung 2016 Dec;18(4):199-208
    Semmelweis University, Department of Psychiatry and Psychotherapy, Budapest, Hungary.
    Anti-NMDAR (N-methyl-D-aspartic acid receptor) encephalitis, first described in 2007, is a rare, autoimmune limbic encephalitis. In half of the cases anti-NMDAR antibodies are paraneoplastic manifestations of an underlying tumor (mostly ovarian teratoma). In the early stage of the disease psychiatric symptoms are prominent, therefore 60-70% of the patients are first treated in a psychiatric department. Read More

    Thymoma-associated multi-organ autoimmunity: A case of graft-versus-host disease-like erythroderma complicated by Good syndrome successfully treated by thymectomy.
    J Dermatol 2017 Mar 3. Epub 2017 Mar 3.
    Department of Dermatology, Hiratsuka City Hospital, Kanagawa, Japan.
    Thymoma-associated multi-organ autoimmunity disease (TAMA) is a rare paraneoplastic disorder, clinicopathologically similar to graft-versus-host disease (GVHD). Many reported cases follow a difficult course; half of them die from serious infectious diseases subsequent to immunosuppression induced by chemotherapy for unresectable thymoma, or intensive therapies including systemic steroids for complicating autoimmune diseases and GVHD-like symptoms. We report a patient whose skin symptoms were improved subsequently to total thymectomy. Read More

    Netrin-1 receptor antibodies in thymoma-associated neuromyotonia with myasthenia gravis.
    Neurology 2017 Mar 1;88(13):1235-1242. Epub 2017 Mar 1.
    From the Laboratorio de Neurología (E.T.-V., T.S., J.J.V., L.B.), Instituto de Investigación Sanitaria La Fe, Hospital Universitario y Politécnico La Fe, CIBERER, Valencia; Servicio de Anatomía Patológica (N.M.), Servicio de Neurofisiología Clínica (M.J.C.), and Servicio de Neurología (T.S., J.J.V., L.B.), Hospital Universitario y Politécnico La Fe, Valencia; Laboratorio de Neurobiología Comparada (A.C.-S., V.H.-P., J.M.G.-V.), Instituto Cavanilles, Universidad de Valencia, CIBERNED; Servicio de Neurología (G.M.), Hospital Central de Asturias, Oviedo, Spain; French Reference Center on Paraneoplastic Neurological Syndrome (B.J., J.H.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, and Institut NeuroMyoGene INSERM U1217/CNRS UMR 5310, Université de Lyon-Université Claude Bernard Lyon 1, France; Laboratori de Neurologia (J.D., F.G.), Institut d´Investigacions Biomèdiques August Pi I Sunyer, CIBERER, Barcelona, Spain; and Department of Neurology (J.D.), University of Pennsylvania, Philadelphia, and Institució Catalana de Recerca i Estudis Avançats, Barcelona, Spain.
    Objective: To identify cell-surface antibodies in patients with neuromyotonia and to describe the main clinical implications.

    Methods: Sera of 3 patients with thymoma-associated neuromyotonia and myasthenia gravis were used to immunoprecipitate and characterize neuronal cell-surface antigens using reported techniques. The clinical significance of antibodies against precipitated proteins was assessed with sera of 98 patients (neuromyotonia 46, myasthenia gravis 52, thymoma 42; 33 of them with overlapping syndromes) and 219 controls (other neurologic diseases, cancer, and healthy volunteers). Read More

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