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primary cns vasculitis

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https://www.readbyqxmd.com/read/28864038/radiologically-isolated-cerebral-amyloid-angiopathy-related-inflammation
#1
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/28758039/primary-angiitis-of-the-center-nervous-system-a-clinical-challenge-diagnosed-postmortem
#2
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
#3
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/28734234/association-of-primary-central-nervous-system-vasculitis-with-the-presence-of-specific-human-leucocyte-antigen-gene-variant
#4
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/28718271/focal-hemispheric-central-nervous-system-vasculitis-an-unusual-form-of-primary-angiitis
#5
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/28516076/primary-central-nervous-system-vasculitis-mimicking-a-cortical-brain-tumor-a-case-report
#6
Joo-Seok Lee, Tae-Young Jung, Kyung-Hwa Lee, Seul-Kee Kim
We report a case of primary central nervous system vasculitis (PCNSV) mimicking a cortical brain tumor. A 25-year-old woman presented with a 2-week history of headache and transient right hemiparesis. Brain magnetic resonance imaging (MRI) revealed a cortical-involving lesion on the left frontal lobe. The 6-cm sized lesion showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The lesion had continual linear enhancement on the subcortical white matter and leptomeninges...
April 2017: Brain Tumor Research and Treatment
https://www.readbyqxmd.com/read/28413538/primary-cns-vasculitis-masquerading-as-glioblastoma-a-case-report-and-review
#7
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/28375836/childhood-primary-large-vessel-cns-vasculitis-single-centre-experience-and-review-of-the-literature
#8
REVIEW
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/28330942/primary-angiitis-of-the-central-nervous-system-magnetic-resonance-imaging-spectrum-of-parenchymal-meningeal-and-vascular-lesions-at-baseline
#9
MULTICENTER STUDY
Grégoire Boulouis, Hubert de Boysson, Mathieu Zuber, Loïc Guillevin, Eric Meary, Vincent Costalat, Christian Pagnoux, Olivier Naggara
BACKGROUND AND PURPOSE: Primary angiitis of the central nervous system remains challenging. To report an overview and pictorial review of brain magnetic resonance imaging findings in adult primary angiitis of the central nervous system and to determine the distribution of parenchymal, meningeal, and vascular lesions in a large multicentric cohort. METHODS: Adult patients from the French COVAC cohort (Cohort of Patients With Primary Vasculitis of the Central Nervous System), with biopsy or angiographically proven primary angiitis of the central nervous system and brain magnetic resonance imaging available at the time of diagnosis were included...
May 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28324788/primary-central-nervous-system-vasculitis-and-its-mimicking-diseases-clinical-features-outcome-comorbidities-and-diagnostic-results-a-case-control-study
#10
J Becker, P A Horn, K Keyvani, I Metz, C Wegner, W Brück, F M Heinemann, J C Schwitalla, P Berlit, M Kraemer
OBJECTIVES: To compare clinical features and outcome, imaging characteristics, biopsy results and laboratory findings in a cohort of 69 patients with suspected or diagnosed primary central nervous system vasculitis (PCNSV) in adults; to identify risk factors and predictive features for PCNSV. PATIENTS AND METHODS: We performed a case-control-study including 69 patients referred with suspected PCNSV from whom 25 were confirmed by predetermined diagnostic criteria based on biopsy (72%) or angiography (28%)...
May 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28217160/primary-angiitis-of-the-central-nervous-system-a-rare-and-reversible-cause-of-childhood-stroke
#11
Aashima Dabas, Sangeeta Yadav
Childhood primary angiitis of the central nervous system (cPACNS) is a rare and a potentially fatal cause of childhood stroke. The disease poses a diagnostic dilemma for the clinicians due to overlapping and varied clinical manifestations such as headache, focal acute neurological deficits, cognitive impairment, or encephalopathy. We report a young boy who presented with low-grade fever and headache but rapidly progressed to develop acute encephalopathy and quadriparesis with multiple cranial nerve palsies, masquerading as acute disseminated encephalomyelitis...
October 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28087351/central-nervous-system-vasculitis-in-adults-an-update
#12
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
#13
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/28034820/mycophenolate-mofetil-as-induction-and-long-term-maintaining-treatment-in-childhood-primary-angiitis-of-the-central-nervous-system
#14
Anna Rosati, Alessandra Cosi, Massimo Basile, Alice Brambilla, Renzo Guerrini, Rolando Cimaz, Gabriele Simonini
OBJECTIVE: To report our single centre experience in treating 4 children affected by childhood primary central nervous system vasculitis (cPACNS) using mycophenolate mofetil (MMF). METHODS: From December 2011 to August 2015, 4 patients (3 males; age range: 9 months-13 years) affected by cPACNS were collected. Enrolled children received the following treatment protocol: acetylsalicylic acid and/or anticoagulant therapy with low molecular weight heparin (LMWH) 100 U/k BID replaced by acenocoumarol; methyl-prednisolone (30mg/kg/day for 3-5 days) followed by prednisone (2mg/kg/day), tapered and discontinued over 7-8 months; MMF used for induction therapy and subsequent maintenance phase (750-1000mg/m(2) BID, half-dose for the first 10-15 days followed by full-dose)...
May 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/27987517/-cerebral-infarction-in-a-patient-with-primary-sjogren-s-syndrome-a-case-report-and-literature-review
#15
D Yang, L Qiao, L D Zhao
Sjogren's syndrome (SS) is a chronic autoimmune disorder characterized by lymphocytes infiltration in the exocrine glands. Central nervous system complications of primary SS are not rare, but ischemic stroke has been rarely reported. Here we report a 43-year-old female with a two-year history of primary SS, presenting with sudden cerebral infarction. Her primary SS was diagnosed on the basis of clinical features, high levels of serum anti-SSA and anti-SSB antibodies, salivary gland secretion evaluation and positive sublingual gland biopsy results...
December 18, 2016: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/27940585/adult-primary-angiitis-of-the-central-nervous-system-isolated-small-vessel-vasculitis-represents-distinct-disease-pattern
#16
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)...
March 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/27884882/lymphomatosis-cerebri-diagnostic-challenges-and-review-of-the-literature
#17
REVIEW
Paul J Lee, Idanis Berrios, Carolina Ionete, Thomas Smith
Lymphomatosis cerebri (LC) is a rare variant of a primary central nervous system non-Hodgkin's lymphoma (PCNSL) characterised by diffuse infiltration of tumour cells throughout the brain parenchyma. We present a 68-year-old immunocompetent woman with headaches, dizziness, blurred vision, localised right leg weakness and rapidly progressive dementia. A brain MRI demonstrated diffuse T2 hyperintense white matter lesions that did not enhance with contrast. The clinical differential diagnosis of these lesions included metastatic disease, infectious or inflammatory process such as sarcoidosis, lymphoma, demyelinating disease and less likely vascular aetiology, such as vasculitis or ischaemic stroke...
November 24, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27745577/-primary-central-nervous-system-vasculitis-in-children
#18
Marinka Twilt, Troels Herlin
In children, inflammatory brain diseases (IBrainDs) are increasingly being recognized. Clinical, neuroimaging and laboratory features are overlapping, and an exact diagnosis can be significantly delayed. Novel antibodies have been discovered and should be included in the diagnostic evaluation. Specific neuroradiological tests such as conventional angiography or vessel wall enhancement can assist in supporting the diagnosis. Brain biopsy should be considered in children with IBrainD with unclear pathology. Treatment should be tailored to the underlying pathogenesis...
October 17, 2016: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/27677285/-clinical-aspects-and-diagnostics-of-cerebral-vasculitis
#19
W Reith, C Kraus, N Harsch
CLINICAL/METHODICAL ISSUE: Vasculitis is a rare cause of diseases of the central nervous system (CNS). Vasculitis can be divided into primary and secondary forms, of which the vast majority can be manifested in various organ systems, including the CNS. Isolated vasculitis of the CNS is limited to the CNS and clinical neurological symptoms as with the other forms of vasculitis, are headaches, encephalopathy, focal deficits and seizures. A criterion of isolated CNS vasculitis is the clinical and laboratory diagnostic exclusion of other forms of vasculitis and the involvement of other organ systems...
October 2016: Der Radiologe
https://www.readbyqxmd.com/read/27570401/primary-cns-vasculitis-presenting-as-intraventricular-bleeding
#20
Sreeja Hareendranathan Pillai, Sapna Erat Sreedharan, Girish Menon, Santhosh Kannoth, Sylaja Pn
Primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Intracranial haemorrhages though less reported are in the form of parenchymal haemorrhage and subarachnoid haemorrhage. We report a case of PACNS with intraventricular haemorrhage due to aneurysms secondary to progression of vasculitis.
July 2016: Annals of Indian Academy of Neurology
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