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https://www.readbyqxmd.com/read/29901574/paraneoplastic-cerebellar-degeneration-associated-with-cerebellar-hypermetabolism-case-report
#1
Ammar Taha Abdullah Abdulaziz, Xiao Qing Yu, Le Zhang, Xin Yue Jiang, Dong Zhou, Jin Mei Li
RATIONALE: Paraneoplastic cerebellar degeneration (PCD) is an immune-mediated neurological deficit affecting the cerebellum. Anti-Yo antibody positive PCD is a rare occurrence most likely associated with gynecologic or breast malignancies. The identification of the underlying tumor is a diagnostic challenge in many of these patients. PATIENT CONCERNS: We present a 68-year-old woman with acute symptoms of PCD as a first sign of underlying occult malignancy. Further investigation revealed a positive anti-Yo antibody...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29862846/multicenter-validation-of-18-f-fdg-pet-and-support-vector-machine-discriminant-analysis-in-automatically-classifying-patients-with-amyotrophic-lateral-sclerosis-versus-controls
#2
Ludovic D'hulst, Donatienne Van Weehaeghe, Adriano Chiò, Andrea Calvo, Cristina Moglia, Antonio Canosa, Angelina Cistaro, Stefanie Ma Willekens, Joke De Vocht, Philip Van Damme, Marco Pagani, Koen Van Laere
OBJECTIVE: 18 F-Fluorodeoxyglucose (18 F-FDG) positron emission tomography (PET) single-center studies using support vector machine (SVM) approach to differentiate amyotrophic lateral sclerosis (ALS) from controls have shown high overall accuracy on an individual patient basis using local a priori defined classifiers. The aim of the study was to validate the SVM accuracy on a multicentric level. METHODS: A previously defined Belgian (BE) group of 175 ALS patients (61...
June 4, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29781194/paraneoplastic-autoimmune-encephalitis-associated-with-pleomorphic-lung-carcinoma-an-autopsy-case-report
#3
Takashi Ando, Yoji Goto, Kazuo Mano, Fumio Nomura, Masako Kurashige, Masafumi Ito, Maya Mimuro, Yasushi Iwasaki, Masahisa Katsuno, Mari Yoshida
A 64-year-old man was admitted with acute onset disturbed consciousness. Cerebrospinal fluid analysis revealed pleocytosis and elevated protein, with negative cultures and PCR. Serum antibodies for autoimmune encephalitis were also negative. Brain magnetic resonance imaging (MRI) was unremarkable, but whole-body CT scan showed a tumor in the left lower lung lobe. Bronchial brush cytology demonstrated clusters of malignant cells, and 18 F-fluorodeoxyglucose positron emission tomography showed multiple lesions and increased uptake in the lung tumor...
May 20, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29780941/unilateral-paraneoplastic-optic-disc-edema-and-retinal-periphlebitis-in-pineal-germinoma
#4
Gunay Uludag, Aslihan Onay, Sumru Onal
Purpose: To describe a unilateral ocular paraneoplastic syndrome in pineal germinoma. Observations: A 24-year-old male presented with diplopia, excessive thirst, and frequent urination. Cranial MRI showed a mass in pineal gland. Dorsal midbrain syndrome signs were present. Examination showed optic disc edema and segmental retinal periphlebitis in right and normal fundus in left eye. Rheumatologic work-up was negative. Brain biopsy confirmed pineal germinoma. Retinal findings were attributed to paraneoplastic syndrome...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29681644/paraneoplastic-lambert-eaton-syndrome-in-a-patient-with-disseminated-metastatic-cancer
#5
Gregorio Arellano-Aguilar, Erik Santiago Núñez-Mojica, José Luis Gutiérrez-Velazco, Luis Gerardo Domínguez-Carrillo
Background: Neurological paraneoplastic syndromes are rare, occur in 0.01% of all cancer patients; like part of them, the Lambert-Eaton syndrome is an autoimmune presynaptic disorder of neuromuscular transmission characterized by muscle weakness and neurovegetative dysfunction, and often associated with small cell lung cancer. Case report: A 72 years old female with a family history of lung cancer and leukemia, with 7 months of dry cough and 3 months with waist and pelvic muscle weakness, oropharyngeal dysphagia, dry mouth, chronic constipation and weight loss of 10 kg...
2018: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/29679372/paraneoplastic-cerebellar-degeneration-yo-antibody-alters-mitochondrial-calcium-buffering-capacity
#6
D Panja, C A Vedeler, M Schubert
AIM: Neurodegeneration is associated with dysfunction of calcium buffering capacity and thereby sustained cellular and mitochondrial calcium overload. Paraneoplastic cerebellar degeneration (PCD), characterized by progressive Purkinje neurone degeneration following paraneoplastic Yo antibody internalization and binding to cerebellar degeneration-related protein CDR2 and CDR2L, has been linked to intracellular calcium homeostasis imbalance due to calbindin D28k malfunction. Therefore, we hypothesized that Yo antibody internalization affects not only calbindin calcium binding capacity, but also calcium-sensitive mitochondrial-associated signalling, causing mitochondrial calcium overload and thereby Purkinje neurone death...
April 21, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29613897/immune-mediated-myelopathies
#7
Dean M Wingerchuk
PURPOSE OF REVIEW: This article reviews the clinical presentation, diagnostic evaluation, and management of immune-mediated myelopathies. RECENT FINDINGS: The discovery of several neural autoantibodies and their antigenic targets has revolutionized the investigation and treatment of immune-mediated myelopathies. Detection of these serologic biomarkers can support or establish a diagnosis of an autoimmune myelopathy, and, in the case of paraneoplastic syndromes, indicate the likely presence of an underlying malignancy...
April 2018: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29594605/autonomic-nervous-system-and-cancer
#8
REVIEW
Marta Simó, Xavier Navarro, Victor J Yuste, Jordi Bruna
The autonomic nervous system (ANS) is the main homeostatic regulatory system of the body. However, this widely distributed neural network can be easily affected by cancer and by the adverse events induced by cancer treatments. In this review, we have classified the ANS complications of cancer into two categories. The first includes direct cancer-related complications, such as primary ANS tumors (pheochromocytoma, paraganglioma or neuroblastoma), as well as autonomic manifestations induced by non-primary ANS tumors (primary brain tumors and metastases)...
March 28, 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/29536122/-principles-of-autoimmune-and-paraneoplastic-encephalitis
#9
REVIEW
C G Bien
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...
March 13, 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29513402/inflammation-in-cns-neurodegenerative-diseases
#10
REVIEW
Jodie Stephenson, Erik Nutma, Paul van der Valk, Sandra Amor
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...
March 7, 2018: Immunology
https://www.readbyqxmd.com/read/29489644/a-case-report-of-limbic-encephalitis-in-a-metastatic-colon-cancer-patient-during-first-line-bevacizumab-combined-chemotherapy
#11
Laura Attademo, Stefano De Falco, Mario Rosanova, Marcello Esposito, Federica Mazio, Francesca Foschini, Antonio Santaniello, Giovanni Fiore, Elide Matano, Fiore Manganelli, Chiara Carlomagno
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...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29460203/time-is-cerebellum
#12
EDITORIAL
Hiroshi Mitoma, Mario Manto, Christiane S Hampe
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. Clinically, some patients show remarkable improvement of severe ataxic symptoms associated with trauma, stroke, metabolism, or immune-mediated cerebellar ataxia (IMCA, e...
February 19, 2018: Cerebellum
https://www.readbyqxmd.com/read/29441072/fatal-necrotizing-encephalopathy-after-treatment-with-nivolumab-for-squamous-non-small-cell-lung-cancer-case-report-and-review-of-the-literature
#13
Markus Leitinger, Mihael V Varosanec, Slaven Pikija, Romana E Wass, Dave Bandke, Serge Weis, Michael Studnicka, Susanne Grinzinger, Mark R McCoy, Larissa Hauer, Johann Sellner
Immune checkpoint inhibitors are antibodies, which enhance cellular and humoral immune responses and are approved for the treatment of various tumors. Immune-related adverse events (irAE) involving different organs and systems are, however, among the side-effects. Recent reports of severe persistent neurological deficits and even fatal cases underpin the need for better understanding of the exact pathomechanisms of central nervous system (CNS) toxicity. To our knowledge, we report the first biopsy-proven case of fatal necrotizing encephalopathy after treatment with nivolumab...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29440480/trismus-caused-by-paraneoplastic-brainstem-encephalitis
#14
Naveed Malek, Maxwell Damian
We discuss the assessment and differential diagnoses of a middle-aged man who presented with trismus, double vision and behavioural problems. MRI scan of the brain was initially normal, but a month later showed high signal in the hippocampal region on fluid attenuated inversion recovery sequence (FLAIR) imaging. We suspected a paraneoplastic brainstem encephalitis because of his smoking history, rapidly progressive symptoms and abnormal brainstem signs. A positron emission tomography-CT scan identified abnormal subcarinal nodes, shown on biopsy to be metastatic small cell lung cancer...
April 2018: Practical Neurology
https://www.readbyqxmd.com/read/29383516/glioblastoma-as-differential-diagnosis-of-autoimmune-encephalitis
#15
Alberto Vogrig, Bastien Joubert, Francois Ducray, Laure Thomas, Cristina Izquierdo, Kévin Decaestecker, Olivier Martinaud, Emmanuel Gerardin, Sylvie Grand, Jérome Honnorat
OBJECTIVE: To identify the clinical and radiological features that should raise suspicion for the autoimmune encephalitis (AE)-like presentation of glioblastoma. METHODS: This is an observational, retrospective case series of patients referred to the French National Reference Center on Paraneoplastic Neurological Diseases for suspected AE (possible, probable or definite, using the 2016 criteria) who later received a final diagnosis of glioblastoma according to 2016 WHO criteria...
March 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29379898/anti-ma-1-and-anti-ma-2-antibodies-in-isolated-fatal-hypothalamitis
#16
Mario Bustos, Hara Berger, Zeina Carolina Hannoush, Alejandro Ayala, Rochelle Freire, Atil Yilmaz Kargi
Lymphocytic hypothalamitis (LHT) is a rare disease characterized by pituitary dysfunction, autonomic instability, metabolic disturbances, and neuropsychiatric disorders. We report the case of a 30-year-old man found to have LHT that progressed despite treatment with methylprednisolone and intravenous immunoglobulin (IVIG). A literature review was conducted to identify prior studies pertaining to LHT. Our patient presented with several weeks of fatigue, cold intolerance, weight loss, confusion, and headache...
February 1, 2018: Journal of the Endocrine Society
https://www.readbyqxmd.com/read/29378289/pnma-family-protein-interaction-network-and-cell-signalling-pathways-implicated-in-cancer-and-apoptosis
#17
REVIEW
Siew Wai Pang, Chandrajit Lahiri, Chit Laa Poh, Kuan Onn Tan
Paraneoplastic Ma Family (PNMA) comprises a growing number of family members which share relatively conserved protein sequences encoded by the human genome and is localized to several human chromosomes, including the X-chromosome. Based on sequence analysis, PNMA family members share sequence homology to the Gag protein of LTR retrotransposon, and several family members with aberrant protein expressions have been reported to be closely associated with the human Paraneoplastic Disorder (PND). In addition, gene mutations of specific members of PNMA family are known to be associated with human mental retardation or 3-M syndrome consisting of restrictive post-natal growth or dwarfism, and development of skeletal abnormalities...
May 2018: Cellular Signalling
https://www.readbyqxmd.com/read/29215577/acute-encephalitis-in-an-adult-with-diffuse-large-b-cell-lymphoma-with-secondary-involvement-of-the-central-nervous-system-infectious-or-non-infectious-etiology
#18
Surinder S Moonga, Kenneth Liang, Burke A Cunha
Both infectious and non-infectious etiologies of acute encephalitis have been described, as well as their specific presentations, diagnostic tests, and therapies. Classic findings of acute encephalitis include altered mental status, fever, and new lesions on neuroimaging or electroencephalogram (EEG). We report an interesting case of a 61-year-old male with a history of diffuse large B-cell lymphoma with secondary involvement of the central nervous system (SCNS-DLBCL). He presented with acute encephalitis: altered mental status, fever, leukocytosis, neuropsychiatric symptoms, multiple unchanged brain lesions on computed tomography scan of the head, and EEG showed mild to moderate diffuse slowing with low-moderate polymorphic delta and theta activity...
December 7, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/29123465/autoimmunity-as-a-driving-force-of-cognitive-evolution
#19
Serge Nataf
In the last decades, increasingly robust experimental approaches have formally demonstrated that autoimmunity is a physiological process involved in a large range of functions including cognition. On this basis, the recently enunciated "brain superautoantigens" theory proposes that autoimmunity has been a driving force of cognitive evolution. It is notably suggested that the immune and nervous systems have somehow co-evolved and exerted a mutual selection pressure benefiting to both systems. In this two-way process, the evolutionary-determined emergence of neurons expressing specific immunogenic antigens (brain superautoantigens) has exerted a selection pressure on immune genes shaping the T-cell repertoire...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29111419/diagnosis-of-multiple-system-atrophy
#20
REVIEW
Jose-Alberto Palma, Lucy Norcliffe-Kaufmann, Horacio Kaufmann
Multiple system atrophy (MSA) may be difficult to distinguish clinically from other disorders, particularly in the early stages of the disease. An autonomic-only presentation can be indistinguishable from pure autonomic failure. Patients presenting with parkinsonism may be misdiagnosed as having Parkinson disease. Patients presenting with the cerebellar phenotype of MSA can mimic other adult-onset ataxias due to alcohol, chemotherapeutic agents, lead, lithium, and toluene, or vitamin E deficiency, as well as paraneoplastic, autoimmune, or genetic ataxias...
May 2018: Autonomic Neuroscience: Basic & Clinical
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