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Cns vasculitis

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https://www.readbyqxmd.com/read/28420057/central-nervous-system-vasculitis-in-systemic-lupus-erythematosus-a-case-series-report-in-a-tertiary-referral-centre
#1
M Rodrigues, O Galego, C Costa, D Jesus, P Carvalho, M Santiago, A Malcata, L Inês
Central nervous system (CNS) vasculitis (CNS) in systemic erythematosus lupus (SLE) is a rare and challenging diagnosis. We report four cases of CNS vasculitis that occurred 5 to 16 years after the diagnosis of SLE. Magnetic resonance imaging (MRI) detected different features suggestive of CNS vasculitis: enhancement and thickening of the vascular wall, vascular stenosis, ischemic brain lesions and intracerebral haemorrhage unlikely to correspond to other mimic aetiologies. Three patients received combination therapy with glucocorticoids (GC) and cyclophosphamide (CYC)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28413538/primary-cns-vasculitis-masquerading-as-glioblastoma-a-case-report-and-review
#2
Pelluru Pavan Kumar, Alugolu Rajesh, Rukmini Mrudula Kandadai, Aniruddh Kumar Purohit, Challa Sundaram
Isolated angitis of the central nervous system (IACNS)/primary angitis of central nervous system vasculitis (PACNS) is an uncommon vascular disease, sparingly presenting as an isolated inflammatory lesion on magnetic resonance imaging (MRI). The disease usually manifests as a long-drawn and progressive ischemic event. Delay in diagnosis due to focal nature of the lesion also contributes to the poor prognosis as the dismal natural history and immunosuppressive therapy. To date, only a few cases with tumor-like isolated angitis of CNS have been reported with clear and definitive diagnostic workup...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28392651/degos-like-lesions-associated-with-systemic-lupus-erythematosus
#3
Min Soo Jang, Jong Bin Park, Myeong Hyeon Yang, Ji Yun Jang, Joon Hee Kim, Kang Hoon Lee, Geun Tae Kim, Hyun Hwangbo, Kee Suck Suh
Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement...
April 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28386527/coccidioidal-meningitis-complicated-by-central-nervous-system-vasculitis-in-a-patient-with-leukemia
#4
Dany Tager, Anne Hatch, Jennifer Segar, Brentin Roller, Mayar Al Mohajer, Tirdad T Zangeneh
Central Nervous System (CNS) vasculitis is the most common life-threatening complication of coccidioidal meningitis. It is manifested by cerebral ischemia, hemorrhage, and infarction. We report a case of CNS vasculitis in a patient receiving chemotherapy and review of the literature on coccidioidal meningitis. The patient was treated with combination antifungal therapy and a short course of high dose corticosteroids with a modest improvement in her neurological examination after initiation of steroids.
June 2017: Medical Mycology Case Reports
https://www.readbyqxmd.com/read/28375836/childhood-primary-large-vessel-cns-vasculitis-single-centre-experience-and-review-of-the-literature
#5
Sonja Walsh, Ralf Knöfler, Gabriele Hahn, Judith Lohse, Reinhard Berner, Sebastian Brenner, Martin Smitka, Maja von der Hagen, Christian M Hedrich
Ischaemic brain injuries are rare conditions in the paediatric age group. Main causes include non-arteriosclerotic arteriopathies, which in childhood usually result from primary vasculitis of large or small vessels and lead to impaired perfusion and subsequent ischaemic brain lesions. In accordance with the nomenclature of systemic forms, CNS vasculitis is subdivided into groups, based on the size of affected vessels: angiography-positive primary angiitis of medium-sized and large vessels (pPACNS), and angiography-negative angiitis of small vessels (svPACNS)...
March 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28368694/autoimmune-mediated-psychosis-a-case-of-susac-syndrome-in-a-drug-user
#6
Pablo Barrio, Mercè Balcells, Delón La Puma, Carles Gaig
OBJECTIVE: Susac syndrome, a rare disorder, is thought to be mediated by autoantibodies. One of the potential targets of these autoantibodies could be an antigen in the microvessels of the brain, the retina, and the inner ear leading to central nervous system (CNS) alterations, visual disturbances, and hearing deficits. Our aim is to expand clinicians' diagnostic options when facing psychosis due to medical conditions. METHODS: A case report was conducted for this study...
April 2017: Journal of Dual Diagnosis
https://www.readbyqxmd.com/read/28355982/low-copy-numbers-of-fcgr3a-and-fcgr3b-associated-with-chinese-patients-with-sle-and-aasv
#7
Y Qi, X Zhou, D Bu, P Hou, J Lv, H Zhang
Low-affinity Fcγ receptors (FcγR) act as key mediators of the pathogenic effects of autoantibodies. In this study, we aimed to determine whether copy number variations (CNVs) in FCGR3A and FCGR3B were associated with systemic lupus nephritis (SLE) and ANCA-associated systemic vasculitis (AASV) in Chinese individuals. A total of 1118 individuals were enrolled, including 415 SLE patients, 139 AASV patients, and 564 healthy controls. FCGR3A and FCGR3B copy numbers (CNs) were determined by both a paralogue ratio test and TaqMan quantitative PCR assay...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28316855/central-nervous-system-involvement-in-henoch-schonlein-purpura-in-children-and-adolescents
#8
Iliyana H Pacheva, Ivan S Ivanov, Krastina Stefanova, Elena Chepisheva, Lyubov Chochkova, Dafina Grozeva, Angelina Stoyanova, Stojan Milenkov, Penka Stefanova, Anna Petrova
Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009-2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28281443/central-nervous-system-tuberculosis
#9
John M Leonard
Central nervous system tuberculosis (CNS-TB) takes three clinical forms: meningitis (TBM), intracranial tuberculoma, and spinal arachnoiditis. TBM predominates in the western world and presents as a subacute to chronic meningitis syndrome with a prodrome of malaise, fever, and headache progressing to altered mentation and focal neurologic signs, followed by stupor, coma, and death within five to eight weeks of onset. The CSF formula typically shows a lymphocytic pleocytosis, and low glucose and high protein concentrations...
March 2017: Microbiology Spectrum
https://www.readbyqxmd.com/read/28240590/a-meta-analysis-of-avascular-necrosis-in-systemic-lupus-erythematosus-prevalence-and-risk-factors
#10
Tatiana Nevskaya, Maeve P Gamble, Janet E Pope
OBJECTIVES: To determine the prevalence of and risk factors for avascular necrosis (AVN) in systemic lupus erythematosus (SLE). METHODS: MEDLINE, CINAHL, Web of Science, EMBASE and Cochrane Library were searched from inception to July, 2015 and a random effects model was used to combine frequencies; study quality was assessed using STROBE. RESULTS: 2,041 citations identified 62 articles. Many results had high heterogeneity. The prevalence of symptomatic AVN was 9% (range 0...
February 24, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28240529/decreased-vessel-wall-enhancement-as-a-biomarker-for-response-to-corticosteroids-in-a-patient-with-cns-vasculitis
#11
Waleed Brinjikji, Vance Lehman, John Huston, Patrick H Luetmer, Giuseppe Lanzino, Alejandro A Rabinstein
No abstract text is available yet for this article.
February 27, 2017: Journal of Neurosurgical Sciences
https://www.readbyqxmd.com/read/28197038/atypical-presentation-of-pheochromocytoma-central-nervous-system-pseudovasculitis
#12
Ketankumar Rupala, Varun Mittal, Rajiv Gupta, Rajiv Yadav
Pheochromocytoma has atypical presentation in 9%-10% of patients. Atypical presentations include myocardial infarction, renal failure, and rarely cerebrovascular events. Various etiologies for central nervous system (CNS) involvement in pheochromocytoma have been described in the literature. A rare association of CNS vasculitis-like features has been described with pheochromocytoma. We report a rare case of pheochromocytoma detected on evaluation for CNS vasculitis-like symptoms.
January 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28174559/zika-virus-chikungunya-virus-and-dengue-virus-in-cerebrospinal-fluid-from-adults-with-neurological-manifestations-guayaquil-ecuador
#13
Nathalie Acevedo, Jesse Waggoner, Michelle Rodriguez, Lissette Rivera, José Landivar, Benjamin Pinsky, Hector Zambrano
Zika virus (ZIKV), chikungunya virus (CHIKV), and dengue virus (DENV) have been associated with clinical presentations that involve acute neurological complaints. In the current study, we identified ZIKV, CHIKV, and DENV in cerebrospinal fluid (CSF) samples from patients admitted to the Hospital Luis Vernaza (Guayaquil, Ecuador) to the Emergency Room or the Intensive Care Unit, with neurological symptoms and/or concern for acute arboviral infections. Viral RNA from one or more virus was detected in 12/16 patients...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28120349/glial-fibrillary-acidic-protein-immunoglobulin-g-as-biomarker-of-autoimmune-astrocytopathy-analysis-of-102-patients
#14
Eoin P Flanagan, Shannon R Hinson, Vanda A Lennon, Boyan Fang, Allen J Aksamit, P Pearse Morris, Eati Basal, Josephe A Honorat, Nora B Alfugham, Jenny J Linnoila, Brian G Weinshenker, Sean J Pittock, Andrew McKeon
OBJECTIVE: A novel autoimmune central nervous system (CNS) disorder with glial fibrillary acidic protein (GFAP)-IgG as biomarker was recently characterized. Here, 102 patients with GFAP-IgG positivity are described. METHODS: The 102 included patients had: (1) serum, cerebrospinal fluid (CSF), or both that yielded a characteristic astrocytic pattern of mouse tissue immunostaining; (2) confirmation of IgG reactive with specific GFAP isoforms (α, ɛ, or κ) by cell-based assays; and (3) clinical data available...
February 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28087351/central-nervous-system-vasculitis-in-adults-an-update
#15
REVIEW
Lívia Almeida Dutra, Alexandre Wagner Silva de Souza, Gabriela Grinberg-Dias, Orlando Graziani Povoas Barsottini, Simone Appenzeller
Primary central nervous system vasculitis (PCNSV) is a challenging diagnosis due to broad clinical manifestations and variable specificity and sensitivity of laboratory and imaging diagnostic tools. Differential diagnosis includes reversible cerebral vasoconstriction syndrome (RCVS), secondary vasculitis of the CNS and other noninflammatory vasculopathies. Brain biopsy is essential for definitive diagnosis and to exclude mimickers. Recent data show that data large-vessel PCNSV present worse prognosis when compared to small-vessel PCNSV...
February 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28074532/pathogenesis-of-developmental-anomalies-of-the-central-nervous-system-induced-by-congenital-cytomegalovirus-infection
#16
REVIEW
Hideya Kawasaki, Isao Kosugi, Shiori Meguro, Toshihide Iwashita
In humans, the herpes virus family member cytomegalovirus (CMV) is the most prevalent mediator of intrauterine infection-induced congenital defect. Central nervous system (CNS) dysfunction is a distinguishing symptom of CMV infection, and characterized by ventriculoencephalitis and microglial nodular encephalitis. Reports on the initial distribution of CMV particles and its receptors on the blood brain barrier (BBB) are rare. Nevertheless, several factors are suggested to affect CMV etiology. Viral particle size is the primary factor in determining the pattern of CNS infections, followed by the expression of integrin β1 in endothelial cells, pericytes, meninges, choroid plexus, and neural stem progenitor cells (NSPCs), which are the primary targets of CMV infection...
February 2017: Pathology International
https://www.readbyqxmd.com/read/28004435/biopsy-proven-case-of-epstein-barr-virus-ebv-associated-vasculitis-of-the-central-nervous-system
#17
Kohei Kano 鹿野耕平, Takayuki Katayama, Shiori Takeguchi, Asuka Asanome, Kae Takahashi, Tsukasa Saito, Jun Sawada, Masato Saito, Ryogo Anei, Kyousuke Kamada, Naoyuki Miyokawa, Hiroshi Nishihara, Naoyuki Hasebe
A 75-year-old woman was admitted to our hospital with rapidly deteriorating consciousness disturbance. She had a 7-year history of rheumatoid arthritis (RA), which had been treated with methotrexate (MTX) and prednisolone. Brain T2-weighted MRI showed diffuse high-intensity lesions in the cerebral subcortical and deep white matter, bilateral basal ganglia and thalamus. A cerebrospinal fluid examination revealed elevated protein levels and positive Epstein-Barr virus (EBV) DNA. Human immunodeficiency virus was negative...
December 22, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/27955815/salmonella-meningitis-associated-with-monocyte-infiltration-in-mice
#18
Timothy J Bauler, Tregei Starr, Toni A Nagy, Sushmita Sridhar, Dana Scott, Clayton W Winkler, Olivia Steele-Mortimer, Corrella S Detweiler, Karin E Peterson
In the current study, we examined the ability of Salmonella enterica serovar Typhimurium to infect the central nervous system and cause meningitis following the natural route of infection in mice. C57BL/6J mice are extremely susceptible to systemic infection by Salmonella Typhimurium because of loss-of-function mutations in Nramp1 (SLC11A1), a phagosomal membrane protein that controls iron export from vacuoles and inhibits Salmonella growth in macrophages. Therefore, we assessed the ability of Salmonella to disseminate to the central nervous system (CNS) after oral infection in C57BL/6J mice expressing either wild-type (resistant) or mutant (susceptible) alleles of Nramp1...
January 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/27940585/adult-primary-angiitis-of-the-central-nervous-system-isolated-small-vessel-vasculitis-represents-distinct-disease-pattern
#19
Hubert de Boysson, Grégoire Boulouis, Achille Aouba, Boris Bienvenu, Loïc Guillevin, Mathieu Zuber, Emmanuel Touzé, Olivier Naggara, Christian Pagnoux
OBJECTIVES: We aimed to identify whether presentations and outcomes in adult patients with isolated small-vessel primary angiitis of the CNS (PACNS) would differ from other patients with large/medium-vessel involvement. METHODS: In the French PACNS cohort, we compared the characteristics, treatments and outcomes of patients with isolated small-vessel disease (normal CT, MR and/or conventional angiograms, brain biopsy positive for vasculitis) with other patients who had large/medium-vessel involvement (vessel abnormalities on CT, MR or conventional angiograms)...
December 10, 2016: Rheumatology
https://www.readbyqxmd.com/read/27818812/cns-vasculitis-associated-with-waldenstr%C3%A3-m-macroglobulinemia
#20
Tanawan Riangwiwat, Chris Y Wu, Alberto S Santos-Ocampo, Randal J Liu, Aaron M McMurtray, Beau K Nakamoto
Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient's mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis...
2016: Case Reports in Neurological Medicine
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