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Cns vasculitis

Khaled Badran, Avinash Kumar Kanodia, Mohamed Abdelsadg, James Galea
No abstract text is available yet for this article.
March 15, 2018: BMJ Case Reports
(no author information available yet)
[This corrects the article DOI: 10.1093/pch/pxx086.079.][This corrects the article DOI: 10.1093/pch/pxx086.079.].
November 2017: Paediatrics & Child Health
Carlo Salvarani, Robert D Brown, Teresa J H Christianson, John Huston, Stephen M Ansell, Caterina Giannini, Gene G Hunder
OBJECTIVES: To record the clinical findings, response to therapy, and course of patients with primary CNS vasculitis (PCNSV) associated with lymphoma. PATIENTS AND METHODS: We reviewed the histories of 936 patients with a diagnosis of any type of vasculitis and lymphoma who were seen at the Mayo Clinic over a 32-year period. Ten patients with both PCNSV and lymphoma were identified. We compared the findings in these 10 patients with those from 158 patients with PCNSV without lymphoma seen over 29 years...
February 2, 2018: Neurology
Leila Chimelli
Acquired and hereditary microangiopathies cause cerebral small vessel diseases (CSVD) that impair cognition. The most frequent is primary angiitis of the CNS (PACNS), whose diagnosis remains challenging, requiring a multidisciplinary approach. Secondary vasculitis, CADASIL, miscellaneous microangiopathies and lymphomas, also cause cognitive impairment. Despite the fact that the need for biopsy has decreased in the era of new neuroimaging methods, biopsies that include small leptomeningeal and parenchymal arterial vessels still remain the gold standard to diagnose PACNS and other CSVD, and to exclude mimics such as infections and malignancies...
October 2017: Dementia & Neuropsychologia
Miramir Aghdashi, Seyed-Mostafa Seidmardani, Sara Vossoughian, Seyed Arman Seyed Mokhtari
Familial Mediterranean fever (FMF) is characterized by recurrent episodes of fever accompanied by serosal, synovial, or cutaneous inflammation. The central nervous system (CNS) is rarely involved in FMF. The CNS involvement includes demyelinating lesions, posterior reversible encephalopathy syndrome, pseudotumor cerebri, optic neuritis, and cerebral vasculitis. Here, we present a 20-year-old man, a known case of FMF with abrupt left-sided hemiparesis. Brain magnetic resonance imaging revealed right periventricular infarction...
2018: Clinical Medicine Insights. Case Reports
Maeng Real Park, Mun Ki Min, Ji Ho Ryu, Dae Sub Lee, Kang Ho Lee
Ulcerative colitis (UC) is a chronic and debilitating disorder, characterized by inflammation of the colonic mucosa. UC can be considered a systemic disorder but UC-related manifestations in the central nervous system (CNS) are quite rare. A 29-year-old man was admitted to the emergency department with repeated generalized tonic-clonic (GTC) type seizures. Based on brain CT, brain metastasis or hemorrhagic infarct was suspected. Diffusion-weighted image of brain MRI showed high signal in the left thalamus and heterogenous enhancement in the right parietal and left frontal lobes...
January 4, 2018: American Journal of Emergency Medicine
Eri Kato, Koichiro Tahara, Haeru Hayashi, Aki Shoji, Hiroaki Mori, Tetsuji Sawada
Central nervous system (CNS) involvement in granulomatosis with polyangiitis (GPA), including pachymeningitis and CNS vasculitis, is uncommon. Although intracerebral hemorrhage (ICH) has been reported in GPA, simultaneous multiple ICH (SMICH) is rare. We describe the case of a 50-year-old woman with a history of a limited form of GPA with chronic pachymeningitis who presented with acute-onset headache accompanied by nausea and vomiting, and who developed consciousness impairment. Computed tomography revealed bilateral subcortical ICH...
December 27, 2017: Internal Medicine
Catherine E Najem, Jason Springer, Richard Prayson, Daniel A Culver, James Fernandez, Jinny Tavee, Rula A Hajj-Ali
BACKGROUND/PURPOSE: Common variable immunodeficiency (CVID) is typically characterized by hypogammaglobulinemia and often but not always recurrent infections. Paradoxically, 8-22% of patients with CVID develop granulomatous disease. Granulomata have been described in many organs including the lungs, skin, liver, spleen, kidneys, eyes, lymph nodes, and intestines. Data about central nervous system (CNS) involvement in CVID are extremely rare. We aim to describe a case series and include an extensive literature review of CNS involvement in CVID to understand the different features and patterns of the disease...
October 20, 2017: Seminars in Arthritis and Rheumatism
Neda Zarghami Esfahani, Daniel M Anderson, Connie Pieper, Harold P Adams
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare diseases characterized by a necrotizing small-vessel vasculitis and circulating ANCA that comprise granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Acute ischemic stroke (AIS) can be a manifestation of central nervous system (CNS) involvement in these diseases. Furthermore, intracerebral hemorrhage (ICH) is a potential complication of these necrotizing vasculitides...
December 2017: ENeurologicalSci
Zhihua Chen, Yifeng Miao, Hui Wu, Ran Wang, Zhiyi Zhou, Shilei Zhang, Longtian Chen, Yongming Qiu
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis occasionally affecting central nervous system (CNS), and GPA patients with initial CNS symptoms are even rarer, whose diagnosis is further confused by an absence of positive antineutrophil cytoplasmic antibody. The authors described the characteristics of antineutrophil cytoplasmic antibody -negative GPA with CNS onset in a patient and discussed on its management, which may contribute to future diagnosis and treatment of patients with similar conditions...
December 12, 2017: Journal of Craniofacial Surgery
J Carnero, C Prieto, L Polledo, F J Martínez-Lobo
Recently, the number of clinical reports of growing pigs showing neurological signs possibly related to viral infections has increased. The objective of this report was to describe two outbreaks of an atypical condition observed in 6- to 7-week-old pigs with a morbidity of 20% and a fatality rate of 60% in two unrelated farms of the same company. During the acute phase of the disease, fever, sudden death, neurological signs, ear necrosis and occasional corneal opacity were observed. Histopathological examination revealed interstitial pneumonia, lymphoid depletion and lymphocytic vasculitis in different organs and mild polioencephalomyelitis suggesting a potential viral infection...
November 16, 2017: Transboundary and Emerging Diseases
Kamble J Harsha, Sujit A Jagtap, Tirur R Kapilamoorthy, Chandrasekharan Kesavadas, Bejoy Thomas, Neelima Radhakrishnan
BACKGROUND: Central nervous system (CNS) vasculitis is an uncommon disease, which is a diagnostic and therapeutic challenge for physicians. Large and medium vessel vasculitis is relatively easy to diagnose by angiogram compared to small vessel vasculitis, where angiograms are often normal; imaging features described till date are sensitive but not specific. PURPOSE: Here, we describe distinct magnetic resonance (MR) imaging features of CNS small vessel vasculitis...
November 2017: Neurology India
Rakesh K Gupta
No abstract text is available yet for this article.
November 2017: Neurology India
Jonathan Graf
Central nervous system (CNS) disease is an uncommon but significant complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and affects 3 primary areas of the CNS: the pituitary, the pachymeninges, and the CNS vasculature. Pituitary disease in uncommon, but hormonal deficiencies can be long lasting even in the face of excellent disease response. Chronic hypertrophic pachymeninigitis occurs in anti-proteinase 3-positive patients with systemic GPA and in anti-myeloperoxidase-positive patients with a milder and more limited form of the disease...
November 2017: Rheumatic Diseases Clinics of North America
Monika Czarnowska-Cubała, Adam Włodarczyk, Joanna Szarmach, Katarzyna Gwoździewicz, Joanna Pieńkowska, Mariusz S Wiglusz, Wiesław Jerzy Cubała, Krzysztof Krysta
BACKGROUND: There is evidence for neurosyphilis being associated with the central nervous system vasculitis involving medium and small vessels. As the hemispheric white matter is the major target of these vascular alterations the white matter axonal and myelination disruption may be observed employing measure for the rate of water molecule diffusion. High apparent diffusion coefficient (ADC) correspond to unimpeded water diffusion and indicating white matter disintegration. CASE REPORTS: In a retrospective study exploringcentral nervous system magnetic resonance (MR) images of two subjects presenting with neurosyphilis the ADC values were found to be increased as related to normal values being accompanied with normal appearing white matter of hemispheres...
September 2017: Psychiatria Danubina
George E Fragoulis, Sophia Lionaki, Aliki Venetsanopoulou, Panayiotis G Vlachoyiannopoulos, Haralampos M Moutsopoulos, Athanasios G Tzioufas
The aims of this study were to estimate the frequency of central nervous system (CNS) involvement in Greek patients with granulomatosis with polyangiitis (GPA) and describe the related clinical characteristics and long-term outcomes of these patients. Medical charts of all ANCA-associated vasculitis patients were retrospectively reviewed, and GPA patients with CNS involvement were identified. Demographics, serological, and clinical features throughout the disease course were recorded. Comparisons of disease characteristics and long-term outcomes were performed between GPA patients with and without CNS involvement...
September 15, 2017: Clinical Rheumatology
Huijun Shen, Jianhua Mao, Qiang Shu, Lizhong Du
BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. Severe central nervous system (CNS) involvement is rare in HSP. CASE CHARACTERISTICS: Three children with features of HSP presented with seizures and CNS dysfunction. OBSERVATION: All three children had abnormalities on neuroimaging; 2 had complete remission but one was left with severe neurological damage. MESSAGE: HSP patients may rarely present with CNS involvement with a prolonged course requiring aggressive treatment...
August 15, 2017: Indian Pediatrics
Rubén S Rosales, Roberto Puleio, Guido R Loria, Salvatore Catania, Robin A J Nicholas
Mycoplasmas of humans and animals are usually associated with respiratory, autoimmune, genital and joint diseases. Human mycoplasmas have also been known to affect the brain. Severe central nervous system (CNS) diseases, such as encephalitis, have been linked to Mycoplasma pneumoniae and ureaplasma infections. Less well known is the sheep and goat pathogen, Mycoplasma agalactiae, which has been found in large quantities in the brain where it may be responsible for non-purulent encephalitis as well as ataxia in young animals...
September 5, 2017: Research in Veterinary Science
Marta Pérez-De-Lis, Soledad Retamozo, Alejandra Flores-Chávez, Belchin Kostov, Roberto Perez-Alvarez, Pilar Brito-Zerón, Manuel Ramos-Casals
Biological drugs are therapies designed to target a specific molecule of the immune system that have been linked with the development of autoimmune diseases. Areas covered: The BIOGEAS Registry currently collects information about nearly 13,000 reported cases of autoimmune diseases developed in patients exposed to biologics, including more than 50 different systemic and organ-specific autoimmune disorders, of which psoriasis (n=6375), inflammatory bowel disease (n=845), demyelinating CNS disease (n=803), interstitial lung disease (n=519) and lupus (n=369) were the most frequently reported...
November 2017: Expert Opinion on Drug Safety
M P Lorenzett, P R Pereira, D M Bassuino, G Konradt, W Panziera, M V Bianchi, F F Argenta, M E Hammerschmitt, R A Caprioli, C S L de Barros, S P Pavarini, D Driemeier
BACKGROUND: Trema micrantha is a tree widely distributed throughout the Americas. The tree produces highly palatable leaves that have been associated with natural poisoning in goats, sheep and horses, in which hepatic necrosis and hepatic encephalopathy have been observed. OBJECTIVES: This study describes malacia and haemorrhage in the central nervous system (CNS) due to T. micrantha consumption, with minimal to absent hepatic lesions. STUDY DESIGN: Retrospective case series...
August 14, 2017: Equine Veterinary Journal
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