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https://www.readbyqxmd.com/read/28212920/neonatal-lupus-follow-up-in-infants-with-anti-ssa-ro-antibodies-and-review-of-the-literature
#1
REVIEW
Antonio Alberto Zuppa, Riccardo Riccardi, Simonetta Frezza, Francesca Gallini, Rita Maria Paola Luciano, Giovanni Alighieri, Costantino Romagnoli, Sara De Carolis
Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur...
February 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28195100/a-54-year-old-male-with-rapidly-progressive-neurologic-syndrome-clinicopathologic-correlation-of-a-rare-diagnosis
#2
Deepti Mutreja, Nikhil Moorchung, Salil Gupta, Rajeev Saxena, Rohini S Doshetty, Bhaskar Nandi
Diagnosis of systemic lupus erythematosus (SLE) as primary presentation with central nervous system involvement as a rapidly progressive neurologic syndrome is extremely rare. We present a rare case of a 54-year-old hypertensive male patient, who presented with a fulminant neurologic syndrome. He presented with cerebellar and meningeal signs, aseptic meningitis and had a rapid downhill course following admission. A postmortem revealed feature of systemic connective tissue fulfilling diagnostic criteria of SLE with lupus cerebritis...
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28179389/homonymous-hemianopia-in-the-primary-antiphospholipid-syndrome
#3
Derek Kwun-Hong Ho, Rishi Ramessur, Mradul Gupta, John P Mathews
A woman aged 26 years was referred by her GP to the eye casualty department with sudden-onset left homonymous hemianopia and right-sided headache. Full ophthalmic examination was normal with the exception of a left homonymous hemianopia confirmed with automated perimetry. Urgent CT imaging revealed a non-haemorrhagic cerebral infarct in the right parieto-occipital region. Subsequent blood tests confirmed a diagnosis of antiphospholipid syndrome with positivity in IgG anticardiolipin antibody, IgG anti-β2-GP1 antibody and the Lupus anticoagulant screen...
February 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28133515/recognition-of-transmembrane-protein-39a-as-a-tumor-specific-marker-in-brain-tumor
#4
Jisoo Park, Hyunji Lee, Quangdon Tran, Kisun Mun, Dohoon Kim, Youngeun Hong, So Hee Kwon, Derek Brazil, Jongsun Park, Seon-Hwan Kim
Transmembrane protein 39A (TMEM39A) belongs to the TMEM39 family. TMEM39A gene is a susceptibility locus for multiple sclerosis. In addition, TMEM39A seems to be implicated in systemic lupus erythematosus. However, any possible involvement of TMEM39A in cancer remains largely unknown. In the present report, we provide evidence that TMEM39A may play a role in brain tumors. Western blotting using an anti-TMEM39A antibody indicated that TMEM39A was overexpressed in glioblastoma cell lines, including U87-MG and U251-MG...
January 2017: Toxicological Research
https://www.readbyqxmd.com/read/28068928/newly-recognized-cerebral-infarctions-on-postmortem-imaging-a-report-of-three-cases-with-systemic-infectious-disease
#5
Sakon Noriki, Kazuyuki Kinoshita, Kunihiro Inai, Toyohiko Sakai, Hirohiko Kimura, Takahiro Yamauchi, Masayuki Iwano, Hironobu Naiki
BACKGROUND: Postmortem imaging (PMI) refers to the imaging of cadavers by computed tomography (CT) and/or magnetic resonance imaging (MRI). Three cases of cerebral infarctions that were not found during life but were newly recognized on PMI and were associated with severe systemic infections are presented. CASE PRESENTATIONS: An 81-year-old woman with a pacemaker and slightly impaired liver function presented with fever. Imaging suggested interstitial pneumonia and an iliopsoas abscess, and blood tests showed liver dysfunction and disseminated intravascular coagulation (DIC)...
January 10, 2017: BMC Medical Imaging
https://www.readbyqxmd.com/read/28065614/cerebral-venous-sinus-thrombosis-presenting-feature-of-systemic-lupus-erythematosus
#6
Rajesh Kumar Singh, Sanjeev Kumar Bhoi, Jayantee Kalita, Usha Kant Misra
BACKGROUND: CVST (cerebral venous sinus thrombosis) may sometimes be associated with autoimmune disorders that require specific treatment. The clinical and magnetic resonance imaging (MRI) findings of systemic lupus erythematosus (SLE) patients with CVST are presented and contrasted with CVST without SLE. METHODS: Consecutive patients with CVST admitted in neurology service during 2012-2016 were included. The diagnosis of CVST was confirmed by MR venography or digital subtraction angiography...
January 5, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28050066/antiphospholipid-syndrome-a-diagnostic-challenge
#7
REVIEW
R S Mallhi, Neerja Kushwaha, T Chatterjee, J Philip
The antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilic disorder that is characterized by thrombosis (venous, arterial and microvascular) and obstetric morbidity due to a diverse family of antibodies against phospholipid-binding proteins present in plasma. The term antiphospholipid antibody is actually a misnomer as the antibodies are not against the phospholipid per se, but target the plasma protein co-factors, which bind to anionic PLs. The exact etiology has not been elucidated and is multifactorial...
December 2016: Medical Journal, Armed Forces India
https://www.readbyqxmd.com/read/28028157/brain-histopathology-in-patients-with-systemic-lupus-erythematosus-identification-of-lesions-associated-with-clinical-neuropsychiatric-lupus-syndromes-and-the-role-of-complement
#8
Daniëlle Cohen, Emilie C Rijnink, Rob J A Nabuurs, Gerda M Steup-Beekman, Maarten J Versluis, Bart J Emmer, Malu Zandbergen, Mark A van Buchem, Cornelia F Allaart, Ron Wolterbeek, Jan A Bruijn, Sjoerd G van Duinen, Tom W J Huizinga, Ingeborg M Bajema
OBJECTIVES: Neuropsychiatric (NP) involvement is a poorly understood manifestation of SLE. We studied post-mortem histopathology in relation to clinical NPSLE syndromes and complement deposition in brains of NPSLE and SLE patients and controls. Furthermore, we investigated the correlation between cerebral post-mortem histopathology and ex vivo 7 T MRI findings in SLE and NPSLE. METHODS: A nationwide search for autopsy material yielded brain tissue from 16 NPSLE and 18 SLE patients...
January 2017: Rheumatology
https://www.readbyqxmd.com/read/27903432/antigen-presentation-for-priming-t-cells-in-central-system
#9
Shaoni Dasgupta, Subhajit Dasgupta
Generation of myelin antigen-specific T cells is a major event in neuroimmune responses that causes demyelination. The antigen-priming of T cells and its location is important in chronic and acute inflammation. In autoimmune multiple sclerosis, the effector T cells are considered to generate in periphery. However, the reasons for chronic relapsing-remitting events are obscure. Considering mechanisms, a feasible aim of research is to investigate the role of antigen-primed T cells in lupus cerebritis. Last thirty years of investigations emphasize the relevance of microglia and infiltrated dendritic cells/macrophages as antigen presenting cells in the central nervous system...
January 2017: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/27878065/corticobulbar-tract-involvement-in-neuropsychiatric-systemic-lupus-erythematosus-a-case-report
#10
Ho Kyun Kim, Mun Han, Hui Joong Lee
A 36-year-old woman, diagnosed with systemic lupus erythematosus (SLE), showed bulbar symptoms including impaired memory, slurred speech and swallowing difficulty 7 days before admission. Magnetic resonance imaging (MRI) showed symmetric confluent hyperintensities in the bilateral cerebral white matter on T2 weighted imaging (T2-WI), extended into the genu of the internal capsule and the crus cerebri of the midbrain. MR spectroscopy showed increased choline and decreased N-acetyl aspartate (NAA) peak and positron emission computed tomography (PET CT) showed decreased fluorodeoxyglucose (FDG) uptake on the lateral portion of the frontal lobe, suggesting demyelination of the white matter...
July 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
https://www.readbyqxmd.com/read/27854095/cerebral-venous-sinus-thrombosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome-with-atypical-antibodies-a-case-report
#11
Tsung-Han Ho, Yu-Wei Hsu, Chih-Wei Wang, Jiunn-Tay Lee, Chi-Hsin Ting, Fu-Chi Yang
BACKGROUND: Although Sjögren's syndrome has been known to complicate with white matter lesions, encephalopathy, or stroke, reports of cerebral venous sinus thrombosis due to Sjögren's syndrome with atypical antibodies are rare. CASE REPORT: A 50-year-old woman was admitted to our neurological ward with nausea and vomiting following acute onset of severe headache in the left occipital region. Brain computed tomography revealed no abnormalities. The patient was fully conscious, with normal cognitive functioning, but exhibited unsteady tandem gait...
June 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/27800096/systemic-lupus-erythematous-revealed-by-cytomegalovirus-infection
#12
Rezgui Amel, Karmani Monia, Mzabi Anis, Ben Fredj Fatma, Laouani Chadia
Cytomegalovirus (CMV) infection have been described as exacerbing systemic lupus erythematous (SLE). The role of CMV in starting off SLE remains object of debate. We report a severe presentation of SLE revealed by CMV infection with hemophogocytic syndrome. A 22 old women without a history of systemic disease developed a cutaneous eruption with fever and myalgia persistant for 2 weeks. Laboratory studies revealed a CMV serology supporting acute CMV infection, with positive antinuclear antidody, anti ds DNA, elevated liver functions tests, pancytopenia...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27759348/mesenteric-vasculitis-in-a-case-of-systemic-lupus-erythematosus
#13
Abhinav Anand, Kruthi Malur, Juhi Kawale, Milind Y Nadkar
We present a case of mesentric vasculitis with systemic lupus erythematosus who relapsed after high dose steroids but achieved subsequent remission after starting pulse cyclophosphamide therapy. 38 years old female who had earlier polyserositis and cerebral venous thrombosis was admitted with provisional diagnosis of SLE and developed acute abdominal pain during hospital stay. She was diagnosed as mesenteric vasculitis and initially responded to pulse methylprednisolone. However, she had relapse which subsequently responded to pulse cyclophosphamide and steroids...
July 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27743753/lupus-anticoagulant-hypoprothrombinemia-syndrome-presenting-with-co-existing-cerebral-venous-thrombosis-and-subdural-hemorrhage
#14
N Bel Feki, S Zayet, I Ben Ghorbel, M-H Houman
The lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) - the association of acquired factor II deficiency and lupus anticoagulant - is a rare disease that may cause a predisposition not only to thrombosis but also to severe bleeding. We are reporting on a 36-year-old female patient presenting with co-existing cerebral venous thrombosis and subdural hemorrhage. The coagulation screening showed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal fibrinogen level and platelet count...
December 2016: Journal des Maladies Vasculaires
https://www.readbyqxmd.com/read/27703087/cerebral-small-vessel-disease-burden-is-increased-in-systemic-lupus-erythematosus
#15
Stewart J Wiseman, Mark E Bastin, Charlotte L Jardine, Gayle Barclay, Iona F Hamilton, Elaine Sandeman, David Hunt, E Nicole Amft, Susan Thomson, Jill F F Belch, Stuart H Ralston, Joanna M Wardlaw
BACKGROUND AND PURPOSE: Systemic lupus erythematosus (SLE) increases stroke risk, but the mechanism is uncertain. This study aimed to determine the association between SLE and features on neuroimaging of cerebral small vessel disease (SVD), a risk factor for stroke. METHODS: Consecutive patients attending a clinic for SLE were recruited. All patients underwent brain magnetic resonance imaging; had blood samples taken for markers of inflammation, endothelial dysfunction, cholesterol, and autoantibodies; and underwent cognitive and psychiatric testing...
November 2016: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27684799/cranial-and-lumbosacral-hypertrophic-pachymeningitis-associated-with-systemic-lupus-erythematosus-a-case-report
#16
Fei Han, Ding-Rong Zhong, Hong-Lin Hao, Wei-Ze Kong, Yi-Cheng Zhu, Hong-Zhi Guan, Li-Ying Cui
BACKGROUND: Hypertrophic pachymeningitis (HP) is a chronic disease characterized by inflammatory hypertrophy and fibrosis of dura mater. It can be divided into cranial and spinal forms depending on the location of the lesion. HP involving 2 separate sites simultaneously is quite uncommon. CASE SUMMARY: This study presents a case of a 49-year-old woman with pathologically confirmed cranial and lumbosacral hypertrophic pachymeningitis associated with systemic lupus erythematosus (SLE), which is a rare etiology of HP...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27617783/-2016%C3%A2-review-on-catastrophic-antiphospholipid-syndrome
#17
REVIEW
Nathalie Costedoat-Chalumeau, Laetitia Coutte, Véronique Le Guern, Nathalie Morel, Gaelle Leroux, Romain Paule, Luc Mouthon, Jean-Charles Piette
The catastrophic antiphospholipid syndrome (CAPS) develops in at least 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. The CAPS is characterized by rapidly-progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. In a few days, the patients develop multiorgan failure with renal insufficiency with severe hypertension, pulmonary, cerebral, cardiac, digestive and/or cutaneous involvement...
December 2016: La Presse Médicale
https://www.readbyqxmd.com/read/27606283/a-comprehensive-rehabilitation-approach-in-a-patient-with-serious-neuropsychiatric-systemic-lupus-erythematosus
#18
Yong Jae Ko, Yang Gyun Lee, Ji Woong Park, Sung Ho Ahn, Jin Myoung Kwak, Yoon-Hee Choi
Neuropsychiatric systemic lupus erythematosus (NPSLE) involves the central and peripheral nervous system in patients with systemic lupus erythematosus (SLE). It is essential to specify the problems faced by patients with NPSLE because it causes diverse disabilities and impairs quality of life. After performing a comprehensive evaluation, tailored management should be provided for the patient's specific problems. We report here the case of a 30-year-old female with SLE who experienced serious neuropsychiatric symptoms cerebral infarction followed by posterior reversible encephalopathy syndrome and peripheral polyneuropathy...
August 2016: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/27489465/evaluation-of-cases-with-cerebral-thrombosis-in-children
#19
Olcay Ünver, Gazanfer Ekinci, Büşra Işın Kutlubay, Thomas Gülten, Sağer Güneş, Nilüfer Eldeş Hacıfazlıoğlu, Dilşad Türkdoğan
AIM: We aimed to evaluate the patients who were followed up in our clinic with a diagnosis of cerebral sinovenous thrombosis in terms of age, sex, clinical findings, etiology, thrombophilic factors, imaging findings, treatment and prognosis. MATERIAL AND METHODS: The files of 11 patients who were followed up in our pediatric neurology clinic with a diagnosis of cerebral thrombosis between 1 December 2010 and 31 December 2014 were retrospectively analyzed. RESULTS: Seven of 11 patients were male (63...
June 2016: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/27488202/rapid-reduction-of-antibodies-and-improvement-of-disease-activity-by-immunoadsorption-in-chinese-patients-with-severe-systemic-lupus-erythematosus
#20
Jinxian Huang, Guoxiang Song, Zhihua Yin, Weizhen He, Lijun Zhang, Weihong Kong, Zhizhong Ye
This was an exploratory analysis comparing the safety and efficacy of immunoadsorption (IAS) combination therapy in severe systemic lupus erythematosus (SLE) receiving corticosteroid pulse and immunosuppressant treatment. Patients enrolled all had predominant organ involvement including proteinuria, thrombocytopenia, pericardial effusion, and cerebral involvement requiring corticosteroid pulse treatment. Fifty-two patients in study group received IAS plus corticosteroid and cyclophosphamide treatment. Fifty-two patients in non-IAS group received corticosteroid and cyclophosphamide treatment...
September 2016: Clinical Rheumatology
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