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central nervous system vasculitis

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https://www.readbyqxmd.com/read/28919362/a-homozygote-trex1-mutation-in-two-siblings-with-different-phenotypes-chilblains-and-cerebral-vasculitis
#1
Rabia Miray Kisla Ekinci, Sibel Balci, Atil Bisgin, Derya Ufuk Altintas, Mustafa Yılmaz
Three prime repair exonuclease 1 degrades single and double stranded DNA with 3'-5' nuclease activity and its mutations are related to type 1 IFN mediated autoinflammation due to accumulated intracellular nucleic acids. To date, several cases of systemic lupus erythematosus, Aicardi-Goutieres syndrome, familial chilblain lupus, retinal vasculopathy-cerebral leukodystrophy have been reported with TREX1 mutations. Chilblain lupus is a skin disease characterized by blue-reddish coloring, swelling or ulcers on acral regions of body such as fingertips, heels, nose and auricles...
September 12, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28914375/central-nervous-system-involvement-in-patients-with-granulomatosis-with-polyangiitis-a-single-center-retrospective-study
#2
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
https://www.readbyqxmd.com/read/28891482/encephalopathy-in-henoch-sch%C3%A3-nlein-purpura
#3
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
https://www.readbyqxmd.com/read/28889017/mycoplasmas-brain-invaders
#4
REVIEW
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
https://www.readbyqxmd.com/read/28864038/radiologically-isolated-cerebral-amyloid-angiopathy-related-inflammation
#5
Dimitri Renard, Anne Wacongne, Eric Thouvenot
In amyloid β-related angiitis of the central nervous system (also called cerebral amyloid angiopathy-related inflammation), cerebral amyloid angiopathy occurs in association with primary vasculitis of small- and medium-sized leptomeningeal and cortical arteries. To avoid brain biopsy, clinicoradiological criteria (including clinical features due to inflammation-related uni/multifocal white matter hyperintensities) for the diagnosis of cerebral amyloid angiopathy-related inflammation have been validated recently...
August 29, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28805273/neurotoxicosis-in-horses-associated-with-consumption-of-trema-micrantha
#6
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 due to T. micrantha consumption, with minimal to absent hepatic lesions. STUDY DESIGN: Retrospective case series...
August 14, 2017: Equine Veterinary Journal
https://www.readbyqxmd.com/read/28804113/case-report-of-a-28-year-old-male-with-the-rapid-progression-of-steroid-resistant-central-nervous-system-vasculitis-diagnosed-by-a-brain-biopsy
#7
Keigo Takahashi, Hideki Sato, Hidenori Hattori, Masaki Takao, Shinichi Takahashi, Norihiro Suzuki
A 28-year-old Japanese male without a significant past medical history presented with new-onset generalized clonic seizure and headache. A brain MRI revealed multiple enhanced lesions on both cerebral hemispheres. Laboratory exams showed no evidence of systemic inflammation or auto-immune antibodies such as ANCAs. Despite four courses of high-dose methylprednisolone pulse therapy and five treatments with plasmapheresis, his symptoms worsened and the MRI lesions progressed rapidly. During these treatments, we performed a targeted brain biopsy, that revealed histological findings consistent with a predominant angiitis of parenchymal and subdural small vessels...
August 11, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28765346/pitfalls-of-pacns-a-rare-case-of-central-nervous-system-vasculitis-associated-with-toxoplasmosis
#8
Hua-Xia Yang, Hong-Rui Liu, Feng-Chun Zhang
No abstract text is available yet for this article.
August 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28758039/primary-angiitis-of-the-center-nervous-system-a-clinical-challenge-diagnosed-postmortem
#9
Bayan Al Share, Ali Zakaria, Evan Hiner, Ziyad Iskenderian, Nader Warra
Primary angiitis of the central nervous system (PACNS) is a rare vasculitis involving medium and small blood vessels of the brain, spinal cord, and meninges, without systemic involvement. The diffuse and patchy nature of its pathology is reflected by a wide spectrum of nonspecific clinical symptoms. Diagnosis is challenging due to lack of defined clinical criteria or specific imaging findings. Specific workup should be done only after exclusion of other etiologies, including infectious, neoplastic, toxic, and other vascular etiologies including systemic vasculitis...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28751515/concordance-of-time-of-flight-mra-and-digital-subtraction-angiography-in-adult-primary-central-nervous-system-vasculitis
#10
H de Boysson, G Boulouis, J-J Parienti, E Touzé, M Zuber, C Arquizan, N Dequatre, O Detante, B Bienvenu, A Aouba, L Guillevin, C Pagnoux, O Naggara
BACKGROUND AND PURPOSE: 3D-TOF-MRA and DSA are 2 available tools to demonstrate neurovascular involvement in primary central nervous system vasculitis. We aimed to compare the diagnostic concordance of vessel imaging using 3D-TOF-MRA and DSA in patients with primary central nervous system vasculitis. MATERIALS AND METHODS: We retrospectively identified all patients included in the French primary central nervous system vasculitis cohort of 85 patients who underwent, at baseline, both intracranial 3D-TOF-MRA and DSA in an interval of no more than 2 weeks and before treatment initiation...
July 27, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28735554/magnetic-resonance-imaging-in-central-nervous-system-vasculitis-in-a-patient-affected-by-crioglobulin-negative-hepatitis-c-virus-infection-a-likely-correlation
#11
Silvia Squarza, Alberto Galli, Maurizio Cariati, Federico Alberici, Valentina Bertolini, Fabio Frediani, Carla Uggetti
A 56-year-old man with behavioural disorders and facial-brachio-crural right hemiparesis presented with a brain lesion studied with computed tomography, magnetic resonance imaging and brain biopsy, leading to the diagnosis of cerebral vasculitis. Hepatitis C virus (HCV) infection in a phase of activity, without cryoglobulins, was also detected. Brain biopsy, laboratory analysis and response to a specific therapy supported the diagnosis of central nervous system vasculitis that was HCV related.
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28734234/association-of-primary-central-nervous-system-vasculitis-with-the-presence-of-specific-human-leucocyte-antigen-gene-variant
#12
Markus Kraemer, Jana Becker, Peter A Horn, Jan Claudius Schwitalla, Kathy Keyvani, Imke Metz, Christiane Wegner, Wolfgang Brück, Marc Schlamann, Falko M Heinemann, Peter Berlit
OBJECTIVES: The etiology and genetic susceptibility of primary central nervous system vasculitis (PCNSV) are still unclear. PATIENTS AND METHODS: We analyzed the DNA of 25 Caucasian patients with PCNSV for human leucocyte antigen genes HLA-A, HLA-B, HLA-DRB1, and HLA-DQB1, respectively. HLA-frequencies of the 25 patients with PCNSV were compared with HLA-frequencies of matched Caucasian controls. RESULTS: No statistically significant associations were found for HLA-B, HLA-DR1 and HLA-DQB1 variant...
September 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28724595/hypertensive-encephalopathy-mimicking-cerebral-vasculitis-with-pontine-oedema-cerebellar-white-matter-lesions-and-multiple-cerebral-infarctions
#13
Daniela Ceccarelli, David Hargroves, Ibrahim Balogun, Thomas Webb
A 47-year-old man with poorly controlled hypertension presented with headaches, right-sided weakness and dysarthria. CT and MRI scans of the brain showed widespread abnormalities including significant pontine oedema, basal ganglia and corona radiata infarctions and cerebellar white matter high signal. Imaging of the intracerebral vasculature also demonstrated wall irregularities. Initially a central nervous system inflammatory disorder was thought to be the most likely diagnosis, possibly acute demyelinating encephalomyelitis or cerebral vasculitis, and the patient was treated with high-dose intravenous steroids...
July 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28718271/focal-hemispheric-central-nervous-system-vasculitis-an-unusual-form-of-primary-angiitis
#14
Saeed Arif, Jahanzeb Liaqat, Khuram Haq Nawaz, Asif Hashmat
No abstract text is available yet for this article.
April 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28709761/central-nervous-system-involvement-in-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-report-of-26-patients-and-review-of-the-literature
#15
REVIEW
Raphaël André, Vincent Cottin, Jean-Luc Saraux, Gilles Blaison, Boris Bienvenu, Pascal Cathebras, Robin Dhote, Aurélie Foucher, Helder Gil, Joëlle Lapoirie, David Launay, Valentine Loustau, François Maurier, Edouard Pertuiset, Thierry Zénone, Jörg Seebach, Nathalie Costedoat-Chalumeau, Xavier Puéchal, Luc Mouthon, Loïc Guillevin, Benjamin Terrier
BACKGROUND: Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA), central nervous system (CNS) manifestations are poorly described. This study aimed to describe CNS involvement in EGPA. PATIENTS AND METHODS: This retrospective, observational, multicenter study included patients with EGPA and CNS involvement affecting cranial nerves, brain and/or spinal cord. We also undertook a systematic literature review...
July 12, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28697838/-hypertension-and-intermittent-convulsions-for-one-month-in-a-school-age-child
#16
Mao-Qiang Tian, Shu-Yi Liu, Juan Li, Xiao-Mei Shu
Childhood polyarteritis nodosa (PAN) is a rare systemic vasculitis and the delayed diagnosis and treatment will cause high incidence of sequelae and high mortality. This article reports a girl with childhood PAN due to posterior reversible encephalopathy syndrome (PRES). The girl aged ten years was admitted to the hospital due to hypertension and convulsions for one month. She had complaints of headache, vomiting, and blurred vision before convulsions. At six months before admission, a mass was observed in the neck...
July 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28694624/neuropsychiatric-manifestations-of-scrub-typhus
#17
REVIEW
Sanjay K Mahajan, Sanyam K Mahajan
Scrub typhus is caused by Orientia tsutsugamushi characterized by focal or disseminated vasculitis and perivasculitis which may involve the lungs, heart, liver, spleen and central nervous system. It was thought to have been eradicated from India. Recently it is being reported from many areas of India. The clinical picture and severity of the symptoms varies widely. The neurological manifestations of scrub typhus are not uncommon but are diverse. Meningoencephalitis is classical manifestation of scrub typhus but cerebellitis, cranial nerve palsies, plexopathy, transverse myelitis, neuroleptic malignant syndrome and Guillan-Barré syndrome are other manifestations reported in literature...
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28667559/vasculitis-in-the-central-nervous-system
#18
Anastasia Bougea, Nikolaos Spantideas
Central nervous system (CNS) vasculitides are a heterogeneous group of disorders characterized by an inflammatory cell infiltration and necrosis of blood vessel walls in the brain, spinal cord, and the meninges. The CNS complications are likely to be fatal without judicious use of immunosuppression; thus, early diagnosis may prevent from damage and disability. This chapter updates our knowledge on CNS vasculitis-related immunological mechanisms, neurological complications, diagnosis, and management.
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28588671/multiple-sclerosis-pathology-diagnosis-and-treatments
#19
Wen-Juan Huang, Wei-Wei Chen, Xia Zhang
Multiple sclerosis (MS) is a complex neurodegenerative disease affecting the central nervous system (CNS). The onset of MS has been typically observed in individuals aged from 20 to 40-years, with the female to male ratio of 1:2. MS appears as abrupt onset of focal sensory disturbances that is accompanied by unilateral painless damage of vision, double vision, limb weakness, unsteadiness of gait, and bowel or bladder symptoms. Whereas the exact etiology of the disease is unknown, observational research has suggested genetic and environment influences through an underlined pathophysiology widely believed to be autoimmune in nature...
June 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28588188/a-young-boy-with-l-asparaginase-induced-seizure
#20
M A Aziz, N K Singh, M H Rahman, M R Khan, A L Kabir, M Begum
L-Asparaginase is a critical component in the treatment of acute lymphoblastic leukemia in children. It is known to cause coagulation abnormalities, thrombosis and hemorrhage in the central nervous system in addition to vasculitis and hypersensitivity reactions. This syndrome generally occurs after a few weeks of therapy and may occur after L-asparaginase therapy is completed. Seizures are uncommon symptoms. We report a case of seizure associated with L-asparaginase therapy but no evidence of hemorrhagic or thrombotic cerebrovascular events, completed in the department of Hematology of Bangabandhu Sheikh Mujib Medical University during March & April 2016...
April 2017: Mymensingh Medical Journal: MMJ
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