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    6203 results match your criteria Paraneoplastic Diseases

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    Syndrome and outcome of antibody-negative limbic encephalitis.
    Eur J Neurol 2018 Apr 18. Epub 2018 Apr 18.
    Neuroimmunology Program Institut d'Investigació Biomèdica August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
    Objective: To report the clinical characteristics of 12 patients with limbic encephalitis (LE) who were antibody-negative after a comprehensive immunological study.

    Methods: Review of clinical records of 163 patients with LE. Immunohistochemistry on rat brain, cultured neurons, and cell-based assays were used to identify neuronal autoantibodies. Read More

    Auto-antibodies against P/Q- and N-type voltage-dependent calcium channels mimicking frontotemporal dementia.
    SAGE Open Med Case Rep 2018 10;6:2050313X17750928. Epub 2018 Apr 10.
    Department of Neurology, The University of Texas Health Science Center at Houston, Houston, TX, USA.
    The behavioral variant of frontotemporal dementia is usually a sporadic and progressive neurodegenerative disorder. Here, we report the subacute onset of a frontotemporal dementia phenotype with a treatable etiology. The patient has a history of rheumatoid arthritis, episcleritis, and thyroid eye disease on immunosuppressive therapy. Read More

    Stauffer's syndrome: A comprehensive review and proposed updated diagnostic criteria.
    Urol Oncol 2018 Apr 12. Epub 2018 Apr 12.
    Department of Medical Oncology, Portuguese Oncology Institute of Porto, Porto, Portugal; Portuguese Genitourinary Group (GPGU), Lisbon, Portugal.
    Background: Stauffer's syndrome corresponds to a set of clinical and analytical changes of paraneoplastic nature firstly recognized more than 50 years ago, in association to renal cell carcinoma. A definitive review including universal diagnostic criteria and updated knowledge since the original description is lacking.

    Basic Procedures: The authors conducted a comprehensive bibliographical review and propose updated diagnostic criteria to standardize diagnosis for clinical practice purposes and avoid misclassification. Read More

    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.
    Hum Genome Var 2018 5;5:18016. Epub 2018 Apr 5.
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA.
    Sensory ataxic neuropathy with dysarthria and ophthalmoparesis (SANDO) is a rare phenotype resulting from pathogenic variants of mitochondrial DNA polymerase gamma (). We modeled a novel 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. Read More

    [Paraneoplastic syndromes in rheumatology].
    Z Rheumatol 2018 Apr 10. Epub 2018 Apr 10.
    Zentrum Innere Medizin, Medizinische Klinik und Poliklinik II, Rheumatologie/Klinische Immunologie, Oberdürrbacher Str. 6, 97080, Würzburg, Deutschland.
    Rheumatic paraneoplastic syndromes are paraneoplastic arthritis, palmar fasciitis and polyarthritis syndrome, remitting seronegative symmetrical synovitis with pitting edema, pancreatic panniculitis with polyarthritis, paraneoplastic vasculitis, cancer-associated myositis, hypertrophic osteoarthropathy (Marie-Bamberger disease) and tumor-induced osteomalacia. Typical clinical manifestations, pathogenesis, prognosis, and treatment of this entity are presented. Knowledge of these disease entities can lead to timely diagnosis of the underlying malignant disease and to a higher probability of a cure. Read More

    Chronic oral lichenoid erosions revealing haematological malignancies.
    J Eur Acad Dermatol Venereol 2018 Apr 6. Epub 2018 Apr 6.
    Dermatology Department, Saint-Louis Hospital, Paris, France.
    Lichen planus is a chronic inflammatory disease of the skin and oral mucosa involving CD8+ T cells (1). Paraneoplastic pemphigus (PNP) is an autoimmune blistering skin disease associated with solid cancer or haematological malignancies (HM). We report 4 patients with long term oral lichenoid erosions leading to the diagnosis of PNP associated with HM. Read More

    [Cerebellar Ataxia and Autoantibodies].
    Brain Nerve 2018 Apr;70(4):371-382
    Tokyo Medical University Hachioji Medical Center, Department of Neurology, Tokyo Medical University Hachioji Medical Center.
    The cerebellum is one of the main targets in the central nervous system for autoimmunity. Immune-mediated cerebellar ataxias include gluten ataxia, GAD antibody-associated cerebellar ataxia, Hashimoto's encephalopathy, and paraneoplastic cerebellar degeneration. Autoimmune cerebellar ataxia may be of either insidious or subacute onset, and vertigo or transient neurological symptoms occur in some patients before the onset of the disease, in contrast to spinocerebellar degeneration. Read More

    [P/Q-type Calcium Channel Antibodies in Lambert-Eaton Myasthenic Syndrome].
    Brain Nerve 2018 Apr;70(4):341-355
    Department of Neurology and Strokology, Nagasaki University Hospital.
    Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease of the neuromuscular junction. Approximately 50-60% of patients with LEMS have a tumor, most often small cell lung cancer (SCLC), making LEMS a paraneoplastic neurological syndrome. In Japan, the clinical picture is a male: female ratio of 3:1; mean age, 62 years (17-80 years); and 61% of LEMS patients have SCLC (SCLC-LEMS), with the remainder of patients having no cancer. Read More

    Paraneoplastic pemphigus without antibodies to desmoglein 1 and 3.
    Dermatol Online J 2018 Feb 15;24(2). Epub 2018 Feb 15.
    Feinberg School of Medicine, Northwestern University, Chicago, Illinois,.
    Paraneoplastic pemphigus is a severe autoimmune blistering disease presenting in the setting of underlying malignancy. Paraneoplastic pemphigus is associated with diffuse painful stomatitis throughout the oral cavity with extension to the lips. The cutaneous findings are varied and have been described as lichenoid, pemphigoid, and targetoid lesions. Read More

    Immune-Mediated Myelopathies.
    Continuum (Minneap Minn) 2018 Apr;24(2, Spinal Cord Disorders):497-522
    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. Read More

    Autoimmune encephalitis and psychiatric disorders.
    Rev Neurol (Paris) 2018 Mar 30. Epub 2018 Mar 30.
    Centre de référence maladies rares 'Syndromes neurologiques paranéoplasiques', hospices civils de Lyon, hôpital neurologique, neurologie B, 69677 Bron, France; INMG (NeuroMyoGene Institut), Inserm U1217/CNRS UMR 5310, 69372 Lyon, France; Université de Lyon - université Claude-Bernard Lyon 1, 69372 Lyon, France.
    Autoimmune encephalitis (AE) refers to a rare, newly described, group of diseases associated with specific circulating autoantibodies directed against neuronal proteins used as biomarkers of the disease. Characterization of the associated autoantibodies present in the patients' cerebrospinal fluid (CSF) and/or sera can differentiate the various AE subgroups, which have specific clinical presentations and prognoses, and is therefore essential for proposing appropriate treatments. As psychiatric symptoms may predominate at the onset or over the course of these diseases, the diagnosis is frequently delayed. Read More

    Immune checkpoint inhibitors in small cell lung cancer.
    J Thorac Dis 2018 Feb;10(Suppl 3):S460-S467
    Department of Hematology and Medical Oncology, Winship Cancer Institute of Emory University, Atlanta, GA, USA.
    Small cell lung cancer (SCLC) is a rapidly progressive cancer that often debilitates patients within months of detection and quickly becomes refractory to the limited options of therapy. While SCLC is not generally considered an immunogenic tumor, clinical experience suggests that patients with robust immune response manifesting as paraneoplastic syndrome are more likely to present with limited stage of the disease and tend to have a better prognosis. Monoclonal antibodies targeting critical negative regulators of immune response, so called immune checkpoints, such as cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) and programmed death 1 (PD-1) have expanded the application of immune-based therapies to increasing number of advanced stage cancers. Read More

    Comorbidity in polymyalgia rheumatica.
    Reumatismo 2018 Mar 27;70(1):35-43. Epub 2018 Mar 27.
    Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds.
    Polymyalgia rheumatica (PMR) is the commonest inflammatory rheumatic disease affecting older people. The current mainstay of treatment is long-term oral glucocorticoid therapy. Management of these patients in clinical practice is often complicated by the presence of comorbidity. Read More

    The real evidence for polymyalgia rheumatica as a paraneoplastic syndrome.
    Reumatismo 2018 Mar 27;70(1):23-34. Epub 2018 Mar 27.
    Institute for Primary Care & Health Sciences, Keele University.
    The aim of this study was to systematically consider the evidence for polymyalgia rheumatica (PMR) as a paraneoplastic disease. A systematic review of Medline and Embase was conducted from their inception to February 2017. Risk of bias was assessed using the Newcastle-Ottawa tool. Read More

    Blistering diseases in the mature patient.
    Clin Dermatol 2018 Mar - Apr;36(2):231-238. Epub 2017 Oct 3.
    Department of Dermatovenereology, University Hospital Center Zagreb and University of Zagreb School of Medicine, Zagreb, Croatia. Electronic address:
    Autoimmune blistering diseases (AIBD) are a group of chronic diseases affecting the skin and mucous membranes, with different presentation, clinical course, histologic and immunopathologic findings, and different therapeutic approach. Blisters develop as a result of autoantibodies directed against distinct adhesion structures within desmosomes or within the basement membrane zone. The most common AIBD that develops in the elderly is bullous pemphigoid (previously also named "pemphigoid senilis"), but mature patients can also present with other AIBD as mucous membrane pemphigoid, epidermolysis bullosa acquisita, paraneoplastic pemphigus, pemphigus vulgaris, pemphigus foliaceus, linear IgA dermatosis, and dermatitis herpetiformis. Read More

    Diagnostic challenges in the mature patient: Growing old gracefully.
    Clin Dermatol 2018 Mar - Apr;36(2):116-127. Epub 2017 Nov 14.
    Dermatology Clinic, Department of Medical Science and Public Health, University of Cagliari, Cagliari, Italy. Electronic address:
    Skin diseases in the elderly are frequent and sometimes misleading, due to age-related changes that alter the clinical presentation. Clinicopathologic correlation is often the clue for distinguishing among various pathologies for which clinical signs are more striking than the histopathologic findings. Pitfalls are always lying in wait to ambush inexperienced dermatologists, but usually the microscope provides subtle hints to solve the quandary. Read More

    Neuropathic Pain in Acute and Subacute Neuropathies: A Systematic Review.
    Pain Physician 2018 Mar;21(2):111-120
    Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, UK.
    Background: Neuropathic pain (NP) is a common symptom caused by lesions or diseases of the somatosensory nervous system. Acute/subacute peripheral neuropathies (APN) are rare, however can be particularly painful.

    Objectives: The aim of this systematic review was to estimate the incidence of NP in APN and overview the various etiologies of such neuropathies. Read More

    Paraneoplastic movement disorders.
    Rev Neurosci 2018 Mar 21. Epub 2018 Mar 21.
    Department of Neurochemistry and Neuropathology, Poznan University of Medical Sciences, 49, Przybyszewskiego Str., 60355 Poznan, Poland.
    Paraneoplastic movement disorders are rare, autoimmune-mediated, nonmetastatic complications of malignant neoplasms. Common paraneoplastic movement disorders include paraneoplastic chorea, dystonia, cerebellar degeneration, different types of encephalitis, opsoclonus-myoclonus syndrome, stiff person syndrome, and neuromyotonia. Syndromes usually develop before tumor diagnosis, have subacute onset, and are associated with serum or cerebrospinal fluid antibodies. Read More

    Paraneoplastic Pemphigus Associated With Castleman Disease Detected by 18F-FDG PET/CT.
    Clin Nucl Med 2018 Mar 20. Epub 2018 Mar 20.
    A 36-year-old woman suffering from oral ulcerations, skin rash, and dyspnea was suspected of having paraneoplastic pemphigus. An F-FDG PET/CT was performed to detect the underlying neoplasms and revealed an F-FDG-avid mass in the lower right retroperitoneum. The mucocutaneous lesions and dyspnea were relieved after the tumor excision, and the pathology indicated Castleman disease. Read More

    Gene Profiling in Patients with Systemic Sclerosis Reveals the Presence of Oncogenic Gene Signatures.
    Front Immunol 2018 6;9:449. Epub 2018 Mar 6.
    Immunology Area, Pediatric Hospital Bambino Gesù, Rome, Italy.
    Systemic sclerosis (SSc) is a rare connective tissue disease characterized by three pathogenetic hallmarks: vasculopathy, dysregulation of the immune system, and fibrosis. A particular feature of SSc is the increased frequency of some types of malignancies, namely breast, lung, and hematological malignancies. Moreover, SSc may also be a paraneoplastic disease, again indicating a strong link between cancer and scleroderma. Read More

    Acute Exudative Polymorphous Vitelliform Maculopathy Syndrome; natural history and evolution of fundal and OCT images over time.
    BMJ Case Rep 2018 Mar 20;2018. Epub 2018 Mar 20.
    Department of Ophthalmology, Mater Misericordiae University Hospital, Dublin, Ireland.
    A 33-year-old man presented with a 10-day history of bilateral blurred vision on a background of a prodromal influenza-like illness. Ocular Coherence Tomography (OCT) and fundal examination coincided with a diagnosis of atypical central serous retinopathy. The patient's symptoms worsened during follow-up, and he was started on steroids. Read More

    Intraductal Tubulopapillary Epithelial Proliferation Associated with Type 1 Autoimmune Pancreatitis.
    J Gastrointestin Liver Dis 2018 Mar;27(1):83-87
    Division of Endoscopy, Shizuoka Cancer Center, Suntogun, Shizuoka, 411-8777, Japan.
    A 70-year-old man was referred to our hospital with exacerbation of diabetes. His blood tests showed elevated levels of serum IgG4 and HbA1c. Computed tomography of the pancreatic body demonstrated a weakly enhanced mass, 2 cm in size, with indistinct borders. Read More

    Effect of low-dose cyclophosphamide, ACTH, and IVIG combination immunotherapy on neuroinflammation in pediatric-onset OMS: A retrospective pilot study.
    Eur J Paediatr Neurol 2018 Mar 5. Epub 2018 Mar 5.
    National Pediatric Myoclonus Center, Orlando, FL, USA; National Pediatric Neuroinflammation Organization, Inc, Orlando, FL, USA. Electronic address:
    Introduction: Flow cytometric cerebrospinal fluid (CSF) lymphocyte subset analysis has improved the diagnosis of neuroinflammation and identified multiple markers of inflammation in opsoclonus-myoclonus syndrome (OMS). The aim of this exploratory, retrospective study was to analyze the effect of immunotherapy on these markers to determine which agents are disease modifying.

    Methods: Cross-sectional immunological observations were made in an IRB-approved case-control study, and patients were treated empirically. Read More

    Clinical Characteristics and Etiology of Bilateral Vestibular Loss in a Cohort from Central Illinois.
    Front Neurol 2018 1;9:46. Epub 2018 Mar 1.
    Illinois Neurologic Institute, University of Illinois College of Medicine, Peoria, IL, United States.
    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. Read More

    [Transthyretin Familial Amyloid Polyneuropathy - Disease Profile of a Multisystem Disorder].
    Dtsch Med Wochenschr 2018 Mar 15;143(6):427-430. Epub 2018 Mar 15.
    Transthyretin-related Familial Amyloid Polyneuropathy (ATTR Amyloidosis, former FAP, here called TTR-FAP) is a rare, progressive autosomal dominant inherited amyloid disease ending fatal within 5 - 15 years after final diagnosis. TTR-FAP is caused by mutations of transthyretin (TTR), which forms amyloid fibrils affecting peripheral and autonomic nerves, the heart and other organs. Due to the phenotypic heterogeneity and partly not specific enough clinical symptoms, diagnosis of TTR-FAP can be complicated. Read More

    Clinical, immunological profile and follow up of patients with pemphigus: A study from India.
    Indian J Dermatol Venereol Leprol 2018 Mar 12. Epub 2018 Mar 12.
    Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
    Background: Pemphigus has a protracted course and multiple factors influence its prognosis. The objective of this study was to describe the epidemiology and clinical profile of pemphigus patients and to study its influence on treatment end points.

    Methods: This was a retrospective chart review done in an Indian tertiary care hospital from December 1991 to December 2013. Read More

    [Principles of autoimmune and paraneoplastic encephalitis].
    Nervenarzt 2018 Mar 13. Epub 2018 Mar 13.
    Epilepsie-Zentrum Bethel, Krankenhaus Mara, Maraweg 17-21, 33617, Bielefeld, Deutschland.
    The paraneoplastic and autoimmune encephalitides are now well-established entities. Detection of neural autoantibodies enables specific diagnoses, provides information on the underlying disease pathophysiology, immunological treatability and the likelihood of a tumor being the underlying cause. This is true for the "high ranking" neural antibodies that have been established in the context of circumscribed clinical images and in consideration of large control groups, have been found in the same way by other laboratories and they respond to immunotherapy. Read More

    Updates in Diagnosis, Management, and Treatment of Neuroblastoma.
    Radiographics 2018 Mar-Apr;38(2):566-580
    From the Department of Radiology and Radiological Science (C.C.S., M.J.E.) and Department of Pediatrics (J.M.K.), Medical University of South Carolina, 96 Jonathan Lucas St, MSC 323, Suite 210, Charleston, SC 29425; and Department of Radiology and Imaging Sciences, Emory University, Atlanta, Ga (A.L.A.).
    Neuroblastoma is an embryonic tumor of the peripheral sympathetic nervous system. It is the most common extracranial solid tumor of childhood and accounts for up to 15% of all pediatric cancer fatalities. The manifestation of neuroblastoma is variable depending on the location of the tumor and on the presence or absence of paraneoplastic syndromes. Read More

    Paraneoplastic Pemphigus Associated with B-cell Chronic Lymphocytic Leukemia Treated with Ibrutinib and Rituximab.
    Intern Med 2018 Mar 9. Epub 2018 Mar 9.
    Department of Hematology, National Cancer Center Hospital, Japan.
    Paraneoplastic pemphigus (PNP) is a severe autoimmune blistering disease associated with an underlying malignancy, and its prognosis is poor. We herein report the first patient with B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL)-associated PNP successfully treated with the Bruton's tyrosine kinase inhibitor ibrutinib and rituximab. Although his PNP lesions did not improve with ibrutinib monotherapy, the combination of ibrutinib and rituximab was effective against B-CLL/SLL-associated PNP. Read More

    Inflammation in CNS neurodegenerative diseases.
    Immunology 2018 Mar 7. Epub 2018 Mar 7.
    Centre for Neuroscience and Trauma, Barts and the Blizard Institute, London, School of Medicine and Dentistry, Queen Mary University of London, London, UK.
    Neurodegenerative diseases, the leading cause of morbidity and disability, are gaining increased attention as they impose a considerable socioeconomic impact, due in part to the ageing community. Neuronal damage is a pathological hallmark of Alzheimer's and Parkinson's diseases, amyotrophic lateral sclerosis, Huntington's disease, spinocerebellar ataxia and multiple sclerosis, although such damage is also observed following neurotropic viral infections, stroke, genetic white matter diseases and paraneoplastic disorders. Despite the different aetiologies, for example, infections, genetic mutations, trauma and protein aggregations, neuronal damage is frequently associated with chronic activation of an innate immune response in the CNS. Read More

    Oral pemphigus.
    G Ital Dermatol Venereol 2018 Mar 6. Epub 2018 Mar 6.
    Dipartimento Chirurgico, Medico, Odontoiatrico e di Scienze Morfologiche con Interesse Trapiantologico, Oncologico e di Medicina Rigenerativa, Università degli Studi di Modena e Reggio Emilia, Modena, Italy.
    Involvement of the oral mucosa in patients affected by Pemphigus Vulgaris (PV), Paraneoplastic, IgA Pemphigus and in some cases of Iatrogenic Pemphigus is common, and precede skin lesions in the majority of cases. Intraepidermal bullae are caused by acantholysis, induced by IgG autoantibodies directed against the desmosomes and the domain of numerous keratinocytes self-antigens desmogleins (namely cadherins), thus supporting the autoimmune nature of the disease. Apoptosis may contribute to the acantholysis. Read More

    Good's Syndrome-Association of the Late Onset Combined Immunodeficiency with Thymoma: Review of Literature and Case Report.
    Iran J Allergy Asthma Immunol 2018 Feb;17(1):85-93
    Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
    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. Read More

    Autoimmune Encephalitis With Multiple Autoantibodies: A Diagnostic and Therapeutic Challenge.
    Neurologist 2018 Mar;23(2):55-59
    Departments of Neurology.
    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. Read More

    Sweet's Syndrome (SS) in the Course of Acute Myeloid Leukaemia (AML).
    Open Access Maced J Med Sci 2018 Jan 13;6(1):105-107. Epub 2018 Jan 13.
    Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior (MVR-Sofia), General Skobelev 79, 1606 Sofia, Bulgaria.
    Firstly described by Robert Douglas Sweet in 1964, febrile neutrophilic dermatosis is a disabling, not only cutaneous disorder, clinically characterised by fever and painful erythematous nodules, with a typical background of neutrophilia. Sweet's syndrome (SS) is a chronic inflammatory reactive disorder of unknown cause and incompletely established pathogenesis, although an interplay between genetic and environmental factors, including infections, is likely to occur. A significant part of cases has been demonstrated to be linked with malignancies, especially in the hematologic setting. Read More

    Pemphigus group: overview, epidemiology, mortality, and comorbidities.
    Immunol Res 2018 Apr;66(2):255-270
    Department of Dermatology, Rambam Health Care Campus, POB 9602, 31096, Haifa, Israel.
    Pemphigus forms a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. This group has a chronic course leading to high morbidity and mortality. It is characterized by the production of pathogenic autoantibodies directed against different proteins of the desmosome, leading histologically to intraepidermal cleavage, and clinically to vesicles and erosions on the epithelium of the mucous membranes and/or the skin. Read More

    Thymoma-associated panencephalitis: a newly emerging paraneoplastic neurologic syndrome.
    Neuroimmunol Neuroinflamm 2017 16;4:117-123. Epub 2017 Jun 16.
    Department of Neurology, NYU Langone Medical Center, New York University School of Medicine, New York, NY 10016, USA.
    Recently, a few case reports of thymoma-associated panencephalitis (TAPE) have brought to light a disease entity that has not been fully characterized. Literature review of TAPE reveals an array of associated neuronal antibodies, with varied responses to thymomectomy with or without immunotherapy. This report describes a case of TAPE and proposes that the GABA receptor antibody is a potential target antigen driving the immune process in this disease entity. Read More

    Time Is Cerebellum.
    Cerebellum 2018 Feb 19. Epub 2018 Feb 19.
    School of Medicine, University of Washington, Seattle, WA, 98109, USA.
    The cerebellum characteristically has the capacity to compensate for and restore lost functions. These compensatory/restorative properties are explained by an abundant synaptic plasticity and the convergence of multimodal central and peripheral signals. In addition, extra-cerebellar structures contribute also to the recovery after a cerebellar injury. Read More

    A novel paraneoplastic syndrome with acquired lipodystrophy and chronic inflammatory demyelinating polyneuropathy in an adolescent male with craniopharyngioma.
    J Pediatr Endocrinol Metab 2018 Mar;31(4):479-483
    Division of Nutrition and Metabolic Diseases and the Center for Human Nutrition, Department of Internal Medicine, UT Southwestern Medical Center, K5.214, 5323 Harry Hines Blvd, Dallas, TX 75390-8537, USA.
    Background: Acquired lipodystrophy, craniopharyngioma and chronic inflammatory demyelinating polyneuropathy (CIDP) are individually rare disorders, and have never before been reported in a single patient.

    Case Presentation: A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year. He was diagnosed with CIDP, craniopharyngioma and acquired lipodystrophy. Read More

    MR Imaging of Atraumatic Muscle Disorders.
    Radiographics 2018 Mar-Apr;38(2):500-522. Epub 2018 Feb 16.
    From the Department of Radiology, UCSD Medical Center, San Diego, Calif (E.S., M.N.P.); Department of Radiology, Philippine Orthopedic Center, Quezon City, Maria Clara Street, Santa Mesa Heights, Quezon City, Metro Manila, Philippines 1100 (D.V.F.); and Department of Radiology, Hospital Pablo Tobón Uribe, Medellín, Colombia (C.M.G.).
    Atraumatic disorders of skeletal muscles include congenital variants; inherited myopathies; acquired inflammatory, infectious, or ischemic disorders; neoplastic diseases; and conditions leading to muscle atrophy. These have overlapping appearances at magnetic resonance (MR) imaging and are challenging for the radiologist to differentiate. The authors organize muscle disorders into four MR imaging patterns: (a) abnormal anatomy with normal signal intensity, (b) edema/inflammation, (c) mass, and (d) atrophy, highlighting each of their key clinical and imaging findings. Read More

    Paraneoplastic Pemphigus in a 34-year-old.
    Dermatol Online J 2017 Dec 15;23(12). Epub 2017 Dec 15.
    New York University Health, New York.
    Paraneoplastic Pemphigus (PNP) is a rare and often fatal autoimmune mucocutaneous blistering disease associated with an underlying malignancy. It is thought to be caused by antibodies to tumor antigenscross-reacting with epithelial antigens, specifically desmosomal and hemidesmosomal antigens. There are at least five clinical morphologic variants of PNP, with the earliest and most consistent finding beingsevere stomatitis. Read More

    Sublamina densa-type linear IgA bullous dermatosis with IgA autoantibodies specific for type VII collagen: a case report and clinicopathological review of 32 cases.
    Dermatol Online J 2017 Nov 15;23(11). Epub 2017 Nov 15.
    Department of Dermatology, Division of Medical Sciences, University of Fukui, Fukui, Japan.
    Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disorder characterized by linear deposits of IgA at the basement membrane zone(BMZ) and/or by circulating IgA anti-BMZ antibodies. Comparing with other immuno-bullous diseases, LABD represents a heterogeneous disease entitywith diversity of pathogenic IgA autoantibodies to different hemidesmosomal antigens and an association with malignancies and occasional drug use. We herein present an 82-year-old Japanese man with LABD, whose indirect immunofluorescence using 1M NaCl-split skin showed positive staining for IgA at the dermal side alone. Read More

    Thyroid carcinoma producing β-human chorionic gonadotropin shows different clinical behavior.
    Pathol Int 2018 Apr 15;68(4):207-213. Epub 2018 Feb 15.
    Department of Pathology, Shandong University School of Basic Medical Sciences, 44#, Wenhua Xi Road, Jinan, Shandong, 250012, PR China.
    Columnar cell variant of papillary thyroid carcinoma (CCV-PTC) is an unusual neoplasm, the clinical behavior of which mainly depends on the encapsulation or infiltration. Patients with extensive extrathyroidal extension usually have an aggressive biological behavior. This study confirmed that beta-human chorionic gonadotropin (β-hCG) secreting invasive CCV-PTC has good prognosis comparing with a cohort of follicular cell differentiated thyroid carcinoma. Read More

    Acute bilateral retina hemorrhages beneath internal limiting membrane: An unusual ophthalmological case report of acute leukemia during complete clinical remission.
    Medicine (Baltimore) 2018 Feb;97(7):e0000
    Department of Ophthalmology, West China Hospital of Sichuan University, Wuhou, Chengdu, Sichuan, China.
    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. Read More

    Branch Duct-type Intraductal Papillary Mucinous Neoplasm Presenting as Paraneoplastic Small Plaque Para-psoriasis.
    Indian Dermatol Online J 2018 Jan-Feb;9(1):40-43
    Department of Pathology, VPS Lakeshore Hospital, Cochin, Kerala, India.
    To present and discuss a novel association between branch duct-type intraductal papillary mucinous neoplasm and paraneoplastic parapsoriasis. We present the case of a middle-aged male presenting with skin lesions that were suggestive of parapsoriasis, resistant to treatment, and in whom a diagnosis of branch-type intraductal papillary mucinous neoplasm of the pancreas was eventually made. A curative Whipple's surgery led to complete resolution of the skin lesions within 3 weeks. Read More

    Metastatic "Burned Out" Seminoma Causing Neurological Paraneoplastic Syndrome-Not Quite "Burned Out".
    Front Neurol 2018 30;9:20. Epub 2018 Jan 30.
    Department of Urology, University of Texas Southwestern Medical Center at Dallas, Dallas, TX, United States.
    A 44-year-old man presented with cerebellar ataxia and limbic encephalitis and was ultimately diagnosed with metastatic germ cell neoplasm resulting from a "burned out" primary testicular tumor. The patient had progressive ataxia, leading to a thorough investigation for infectious, autoimmune, metabolic, and malignant causes of acquired cerebellar ataxia that revealed no significant findings. Testicular sonography demonstrated a possible right testicular lesion that was not confirmed on radical inguinal orchiectomy. Read More

    Overexpression of β1 integrin contributes to polarity reversal and a poor prognosis of breast invasive micropapillary carcinoma.
    Oncotarget 2018 Jan 30;9(4):4338-4353. Epub 2017 Nov 30.
    Department of Breast Cancer Pathology and Research Laboratory, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, China.
    Invasive micropapillary carcinoma (IMPC) of the breast is a highly aggressive breast cancer. Polarity reversal exemplified by cluster growth is hypothesized to contribute to the invasiveness and metastasis of IMPC. In this study, we demonstrate that levels of β1 integrin and Rac1 expression were greater in breast IMPC than in invasive breast carcinoma of no specific type and paraneoplastic benign breast tissue. Read More

    Idiopathic Thrombocytopenia Purpura Masking Hodgkin Disease: A Paraneoplastic Syndrome or Simply a Mere Association?
    Case Rep Oncol 2017 Sep-Dec;10(3):1116-1120. Epub 2017 Dec 19.
    Department of Hematology/Oncology, McLaren Oakland Hospital, Michigan State University College of Osteopathic Medicine, Pontiac, Michigan, USA.
    We report a 74-year-old female who presented to the emergency department complaining of bruising and stroke-like symptoms. She underwent a negative stroke work-up but was found to have profoundly low platelets and splenomegaly on examination. An abdominal CT scan was ordered, showing pelvic lymphadenopathy. Read More

    Rare and changeable as a chameleon: paraneoplastic syndromes in renal cell carcinoma.
    World J Urol 2018 Feb 10. Epub 2018 Feb 10.
    Department of Urology, Eberhard Karls University Tübingen, Hoppe-Seyler-Straße 3, 72076, Tübingen, Germany.
    Introduction: Paraneoplastic syndromes (PNS) in renal cell carcinoma (RCC) are important to be recognized by the treating physician, because they may lead to diagnosis of underlying malignant disease. On the other hand, PNS may dominate the clinical picture and can hide the true disorder like a chameleon. When realized, a PNS can be used as a 'neoplastic tumour marker', especially in case of recurrence. Read More

    [Digital ischemia revealing multiple myeloma].
    J Med Vasc 2018 Feb 29;43(1):61-64. Epub 2017 Dec 29.
    Service de médecine interne, CHU Hassan II, Fès, Maroc.
    Digital ulcers generally arise in a context of microangiopathy-related focal ischemia. In women, connective tissue diseases are the main etiology, while in men the cause is often diffuse arterial disease, e.g. Read More

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