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Clinically isolated syndrome

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https://www.readbyqxmd.com/read/29667271/syndrome-and-outcome-of-antibody-negative-limbic-encephalitis
#1
Francesc Graus, Domingo Escudero, Laura Oleaga, Jordi Bruna, Alberto Villarejo-Galende, Jordi Ballabriga, María Inés Barceló, Francisco Gilo, Stoyan Popkirov, Pavel Stourac, Josep Dalmau
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. Patients were included if 1) there was adequate clinical, CSF, and MRI information to classify the syndrome as LE, 2) MRI images were accesible for central review, and 3) serum and CSF were available and confirmed negative for neuronal antibodies...
April 18, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29665046/intrathecal-igm-production-a-promising-high-risk-marker-in-cis-patients
#2
André Huss, Ahmed Abdelhak, Steffen Halbgebauer, Benjamin Mayer, Makbule Senel, Markus Otto, Hayrettin Tumani
Markers predicting the course of patients with clinically isolated syndrome (CIS) towards multiple sclerosis (MS) are urgently needed. We evaluated the predictive values of an intrathecal IgG and IgM production with different methods towards conversion to definite MS according to revised McDonald 2010 criteria in a cohort of 126 CIS patients. An intrathecal IgM production showed the highest likelihood ratio for the conversion of CIS patients to definite MS of 6.3, whereas it was 1.4 for oligoclonal IgG bands...
April 17, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29664509/congenital-hydrocephalus-g%C3%A3-mez-l%C3%A3-pez-hern%C3%A3-ndez-syndrome-an-underdiagnosed-syndrome-a-clinical-case
#3
Camila Gálvez V, Isidro Huete, Marta Hernández
INTRODUCTION: Hydrocephalus is defined as complex conditions influenced by genetic and environmental factors. Excluding hydrocephalus acquired from infection or brain tumors, congenital hydrocephalus with a genetic cause may occur isolated (hydrocephalus isolated, pure or non-syndromatic) or as a component of a genetic syndrome (syndromic hydrocephalus). OBJECTIVE: To present a syndromic congenital hydrocephalus with a known diagnosis, in order to be considered in the study of this pathology and to perform a review of hydrocephaly with a genetic cause...
February 2018: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/29664014/rip-kinase-1-dependent-endothelial-necroptosis-underlies-systemic-inflammatory-response-syndrome
#4
Matija Zelic, Justine E Roderick, Joanne A O'Donnell, Jesse Lehman, Sung Eun Lim, Harish P Janardhan, Chinmay M Trivedi, Manolis Pasparakis, Michelle A Kelliher
Receptor interacting protein kinase 1 (RIPK1) has important kinase-dependent and kinase-independent scaffolding functions that activate or prevent apoptosis or necroptosis in a cell context-dependent manner. The kinase activity of RIPK1 mediates hypothermia and lethality in a mouse model of TNF-induced shock, reflecting the hyperinflammatory state of systemic inflammatory response syndrome (SIRS), where the proinflammatory "cytokine storm" has long been viewed as detrimental. Here, we demonstrate that cytokine and chemokine levels did not predict survival and, importantly, that kinase-inactive Ripk1D138N/D138N hematopoietic cells afforded little protection from TNF- or TNF/zVAD-induced shock in reconstituted mice...
April 16, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29663509/patients-with-a-previous-total-hip-replacement-experience-less-reduction-of-back-pain-following-lumbar-back-surgery
#5
Ted Eneqvist, Erik Bülow, Szilárd Nemes, Helena Brisby, Göran Garellick, Peter Fritzell, Ola Rolfson
The coexistence of degenerative disorders from the hip joint and the lumbar spine, known as "the hip-spine syndrome", is a common encounter in clinical practice. These degenerative conditions may cause similar symptoms which often entail diagnostic challenges in determining the origin of pain. Low back surgery (LBS) with fusion and/or decompression, and total hip replacement (THR) are both often successful interventions. However, the knowledge is limited about the post-operative patient-reported outcome (PRO) following LBS in the presence of a prior THR...
April 16, 2018: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
https://www.readbyqxmd.com/read/29661262/the-genetics-and-molecular-biology-of-fever-associated-seizures-or-epilepsy
#6
Hao Deng, Wen Zheng, Zhi Song
Fever-associated seizures or epilepsy (FASE) is primarily characterised by the occurrence of a seizure or epilepsy usually accompanied by a fever. It is common in infants and children, and generally includes febrile seizures (FS), febrile seizures plus (FS+), Dravet syndrome (DS) and genetic epilepsy with febrile seizures plus (GEFSP). The aetiology of FASE is unclear. Genetic factors may play crucial roles in FASE. Mutations in certain genes may cause a wide spectrum of phenotypical overlap ranging from isolated FS, FS+ and GEFSP to DS...
April 17, 2018: Expert Reviews in Molecular Medicine
https://www.readbyqxmd.com/read/29659729/t-helper-17-1-cells-associate-with-multiple-sclerosis-disease-activity-perspectives-for-early-intervention
#7
Jamie van Langelaar, Roos M van der Vuurst de Vries, Malou Janssen, Annet F Wierenga-Wolf, Isis M Spilt, Theodora A Siepman, Wendy Dankers, Georges M G M Verjans, Helga E de Vries, Erik Lubberts, Rogier Q Hintzen, Marvin M van Luijn
Interleukin-17-expressing CD4+ T helper 17 (Th17) cells are considered as critical regulators of multiple sclerosis disease activity. However, depending on the species and pro-inflammatory milieu, Th17 cells are functionally heterogeneous, consisting of subpopulations that differentially produce interleukin-17, interferon-gamma and granulocyte macrophage colony-stimulating factor. In the current study, we studied distinct effector phenotypes of human Th17 cells and their correlation with disease activity in multiple sclerosis patients...
April 5, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29657682/the-relationship-between-prevalence-of-antibiotics-resistance-and-virulence-factors-genes-of-mrsa-and-mssa-strains-isolated-from-clinical-samples-west-iran
#8
Mohammad Reza Arabestani, Sahar Rastiyani, Mohammad Yousef Alikhani, Seyed Fazlullah Mousavi
Objectives: We sought to evaluate the relationship between the prevalence of antibiotics resistance and virulence factors genes in methicillin-resistant Staphylococcal aureus (MRSA) and methicillin-sensitive S. aureus (MSSA) strains from clinical samples taken in west Iran. Methods: We performed a cross-sectional study using 100 MRSA and 100 MSSA samples isolated from clinical specimens. We used biochemical methods to identify the isolates, which were confirmed by the polymerase chain reaction (PCR) assay...
March 2018: Oman Medical Journal
https://www.readbyqxmd.com/read/29653437/early-infectious-exposures-are-not-associated-with-increased-risk-of-pediatric-onset-multiple-sclerosis
#9
Leena Suleiman, Emmanuelle Waubant, Gregory Aaen, Anita Belman, Leslie Benson, Meghan Candee, Tanuja Chitnis, Mark Gorman, Manu Goyal, Benjamin Greenberg, Yolanda Harris, Janace Hart, Ilana Kahn, Lauren Krupp, Timothy Lotze, Soe Mar, Manikum Moodley, Jayne Ness, Bardia Nourbakhsh, Mary Rensel, Moses Rodriguez, John Rose, Jennifer Rubin, Teri Schreiner, Jan-Mendelt Tillema, Amy Waldman, Bianca Weinstock-Guttman, T Charles Casper, Michael Waltz, Jennifer S Graves
OBJECTIVE: We sought to determine if early infectious exposures such as daycare, early use of antibiotics, vaccinations and other germ exposures including pacifier use and playing on grass are associated with multiple sclerosis (MS) risk in children. METHODS: This was a case-control study of children with MS or clinically isolated syndrome (CIS) and healthy controls enrolled at sixteen clinics participating in the US Network of Pediatric MS Centers. Parents completed a comprehensive environmental questionnaire that captured early infectious exposures, habits, and illnesses in the first five years of life...
March 26, 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29652578/mr-imaging-of-the-fetal-face-comprehensive-review
#10
Murali Nagarajan, Kedar G Sharbidre, Sudeep H Bhabad, Sharon E Byrd
The human face is a complex anatomic structure with an equally complex embryologic development. Derangement of the developmental process can result in various structural anomalies, which range from a mainly cosmetic deformity, such as cleft lip, to potentially life-threatening conditions such as arhinia. These anomalies (a) can occur as isolated anomalies; (b) can be associated with intracranial, spinal, or dental anomalies; or (c) can be a part of various syndromes, thus serving as diagnostic clues in such cases...
April 13, 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29650544/evaluation-and-management-of-right-sided-heart-failure-a-scientific-statement-from-the-american-heart-association
#11
REVIEW
Marvin A Konstam, Michael S Kiernan, Daniel Bernstein, Biykem Bozkurt, Miriam Jacob, Navin K Kapur, Robb D Kociol, Eldrin F Lewis, Mandeep R Mehra, Francis D Pagani, Amish N Raval, Carey Ward
BACKGROUND AND PURPOSE: The diverse causes of right-sided heart failure (RHF) include, among others, primary cardiomyopathies with right ventricular (RV) involvement, RV ischemia and infarction, volume loading caused by cardiac lesions associated with congenital heart disease and valvular pathologies, and pressure loading resulting from pulmonic stenosis or pulmonary hypertension from a variety of causes, including left-sided heart disease. Progressive RV dysfunction in these disease states is associated with increased morbidity and mortality...
April 12, 2018: Circulation
https://www.readbyqxmd.com/read/29645356/patients-with-a-previous-total-hip-replacement-experience-less-reduction-of-back-pain-following-lumbar-back-surgery
#12
Ted Eneqvist, Erik Bülow, Szilárd Nemes, Helena Brisby, Göran Garellick, Peter Fritzell, Ola Rolfson
The coexistence of degenerative disorders from the hip joint and the lumbar spine, known as "the hip-spine syndrome", is a common encounter in clinical practice. These degenerative conditions may cause similar symptoms which often entail diagnostic challenges in determining the origin of pain. Low back surgery (LBS) with fusion and/or decompression, and total hip replacement (THR) are both often successful interventions. However, the knowledge is limited about the post-operative patient-reported outcome (PRO) following LBS in the presence of a prior THR...
April 12, 2018: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
https://www.readbyqxmd.com/read/29626346/propofol-for-implantable-cardioverter-defibrillator-implantation-in-patients-with-brugada-syndrome
#13
Hye-Mee Kw On, Sung-Hoon Kim, Se-Ung Pa Rk, Jin-Ho Rh Im, Hee-Su N Park, Wook-Jong Kim, Gi-Byou Ng Nam
AIMS: Avoiding propofol in patients with Brugada syndrome has been suggested because of the theoretical risk of provoking ventricular arrhythmias, although propofol may be selected for conscious sedation during electrophysiological procedures in catheterization laboratories. This study aimed to document peri-procedural electrocardiographic changes and adverse events in patients with Brugada syndrome undergoing implantable cardioverter defibrillator (ICD) implantation using propofol sedation...
April 6, 2018: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29623865/-stroke-mimicking-acute-peripheral-vertigo-how-to-avoid-misdiagnosis-importance-of-alarm-symptoms-and-assessment-of-eye-movements
#14
R B van Leeuwen, E van Wensen, B R Schudel
Identifying the occurrence of stroke in patients with isolated vertigo is difficult for general practitioners (GPs) as well as for physicians working in the Emergency Department. Three patients with acute vestibular syndrome are presented in this clinical lesson. The importance of an adequate neuro-otological examination is emphasised. However, many GPs lack knowledge of the nystagmus and the head impulse test. Alternative strategies for GPs are discussed.
2018: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/29622867/autologous-stem-cell-transplantation-for-multiple-myeloma-single-centre-experience-from-north-india
#15
Pankaj Malhotra, Uday Yanamandra, Alka Khadwal, Gaurav Prakash, Deepesh Lad, Arjun D Law, Harshit Khurana, M U S Sachdeva, Praveen Bose, Reena Das, Neelam Varma, Subhash Varma
Autologous stem cell transplantation (ASCT) is considered as standard of care in patients with multiple myeloma (MM) patients aged 65 years or younger. We analyzed data of 94 patients of plasma cell dyscrasias who underwent 95 autologous transplants at our institute from October 2003 to Aug 2016. Other than 76 patients of newly diagnosed multiple myeloma, we also transplanted two patients of POEMS syndrome, two patients of plasma cell leukemia, three patients of concurrent light chain deposition disease, three patients of multifocal plasmacytomas, and eight patients of isolated light chain myeloma...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29622363/overlap-between-functional-abdominal-pain-disorders-and-organic-diseases-in-children
#16
A H Langshaw, J M Rosen, L Pensabene, O Borrelli, S Salvatore, N Thapar, D Concolino, M Saps
Functional abdominal pain disorders are highly prevalent in children. These disorders can be present in isolation or combined with organic diseases, such as celiac disease and inflammatory bowel diseases. Intestinal inflammation (infectious and non-infectious) predisposes children to the development of visceral hypersensitivity that can manifest as functional abdominal pain disorders, including irritable bowel syndrome. The new onset of irritable bowel syndrome symptoms in a patient with an underlying organic disease, such as inflammatory bowel disease, is clinically challenging, given that the same symptomatology may represent a flare-up of the inflammatory bowel disease or an overlapping functional abdominal pain disorder...
April 2, 2018: Revista de Gastroenterología de México
https://www.readbyqxmd.com/read/29618723/enhanced-protection-in-mice-induced-by-immunization-with-inactivated-whole-viruses-compare-to-spike-protein-of-middle-east-respiratory-syndrome-coronavirus
#17
Yao Deng, Jiaming Lan, Linlin Bao, Baoying Huang, Fei Ye, Yingzhu Chen, Yanfeng Yao, Wenling Wang, Chuan Qin, Wenjie Tan
The persistent public health threat of infection with Middle East respiratory syndrome coronavirus (MERS-CoV) highlights the need for an effective and safe MERS-CoV vaccine. In this study, we prepared and vaccinated mice with either a Spike (S) protein or inactivated whole MERS-CoV (IV) with a combined adjuvant (alum+CpG) as a vaccine formulation. Similar levels of the anti-S protein IgG response and neutralizing activity were induced by both the S protein and IV vaccines. In addition, immune responses against three other structural proteins, the envelope (E), membrane (M), and nucleocapsid (N) proteins, were also detected in sera of mice that received IV...
April 4, 2018: Emerging Microbes & Infections
https://www.readbyqxmd.com/read/29618063/endoscopic-port-access-surgery-for-isolated-atrioventricular-valve-endocarditis
#18
Johan van der Merwe, Filip Casselman, Bernard Stockman, Apostolos Roubelakis, Yvette Vermeulen, Ivan Degrieck, Frank Van Praet
OBJECTIVES: Our goal was to report the clinical and echocardiographic outcomes of endoscopic port access surgery for isolated active and convalescent atrioventricular valve endocarditis (AVVE). METHODS: Our current surgical team performed endoscopic port access surgery in 66 consecutive patients with isolated AVVE (mean age, 65.5 ± 12.7 years, 37.9% women, mean EuroSCORE II 31.2 ± 24.9%, 45.5% prosthetic AVVE, Staphylococcus aureus 22.2%), between 1 May 2004 and 31 July 2015...
April 2, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29615986/whole-genome-sequencing-and-multiplex-qpcr-methods-to-identify-campylobacter-jejuni-encoding-cst-ii-or-cst-iii-sialyltransferase
#19
Jason M Neal-McKinney, Kun C Liu, Karen C Jinneman, Wen-Hsin Wu, Daniel H Rice
Campylobacter jejuni causes more than 2 million cases of gastroenteritis annually in the United States, and is also linked to the autoimmune sequelae Guillan-Barre syndrome (GBS). GBS often results in flaccid paralysis, as the myelin sheaths of nerve cells are degraded by the adaptive immune response. Certain strains of C. jejuni modify their lipooligosaccharide (LOS) with the addition of neuraminic acid, resulting in LOS moieties that are structurally similar to gangliosides present on nerve cells. This can trigger GBS in a susceptible host, as antibodies generated against C...
2018: Frontiers in Microbiology
https://www.readbyqxmd.com/read/29615267/a-12-month-study-of-the-hikikomori-syndrome-of-social-withdrawal-clinical-characterization-and-different-subtypes-proposal
#20
Ángeles Malagón-Amor, Luis Miguel Martín-López, David Córcoles, Anna González, Magda Bellsolà, Alan R Teo, Víctor Pérez, Antoni Bulbena, Daniel Bergé
Social withdrawal is a new mental health problem increasingly common, present in different cultures, whose psychopathology and treatment is not yet established. This study aims to determine the socio-demographic and clinical features and possible clinical subtypes that predict the 12-month outcomes of cases with hikikomori syndrome, a severe form of social withdrawal. Socio-demographic and clinical data at baseline were analysed as well as data obtained for 12 months after at-home treatment in 190 cases. The inclusion criteria were: spending all time at home, avoiding social situations and relationships, significant deterioration due to social isolation, with a minimum duration of 6 months...
March 23, 2018: Psychiatry Research
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