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central nervous system vasculitis

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https://www.readbyqxmd.com/read/28337860/clinical-features-and-long-term-outcomes-of-105-granulomatosis-with-polyangiitis-patients-a-single-center-experience-from-north-india
#1
Aman Sharma, Godasi S R S N K Naidu, Manish Rathi, Roshan Verma, Manish Modi, Benzeeta Pinto, Kusum Sharma, Varun Dhir, Manphool Singhal, Mahesh Prakash, Ritambhra Nada, Naresh K Panda, Ranjana W Minz
AIM: To describe the clinical features, treatment and long-term outcomes in north Indian patients with granulomatosis with polyangiitis (GPA). METHODS: Clinical details, Birmingham Vasculitis Activity Score (BVAS-v3), laboratory data, histopathology findings, treatment details and outcomes of all consecutive patients diagnosed as having GPA between April 2005 and April 2016 were retrieved. European Vasculitis Study Group definitions were used to classify patients into localized, early systemic, generalized, severe and refractory categories...
March 24, 2017: International Journal of Rheumatic Diseases
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
#2
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...
March 22, 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
#3
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%)...
March 8, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28316855/central-nervous-system-involvement-in-henoch-schonlein-purpura-in-children-and-adolescents
#4
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
#5
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/28273603/rare-presentation-of-wegener-s-granulomatosis-in-the-pituitary-gland-case-report-and-literature-review
#6
Samantha M Baird, Upasna Pratap, Catriona McLean, Candice P Law, Nicholas Maartens
INTRODUCTION: Wegener's granulomatosis (WG) is a systemic vasculitis that can affect a variety of organs including ear, nose and throat, lungs and kidneys. However WG is unusual in the pituitary and rare in the central nervous system. PRESENTATION OF CASE: A 56-year-old male with likely WG presented with polyuria and polydipsia despite six months of conservative medical management. MRI scanning revealed an enlarging heterogeneously enhancing pituitary gland. Following endoscopic transsphenoidal pituitary biopsy and debulking, final tissue pathology was diagnostic for WG in the pituitary gland...
February 20, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28217160/primary-angiitis-of-the-central-nervous-system-a-rare-and-reversible-cause-of-childhood-stroke
#7
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/28197038/atypical-presentation-of-pheochromocytoma-central-nervous-system-pseudovasculitis
#8
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/28175524/354%C3%A2-diagnostic-utility-of-cerebral-biopsy-following-suggestive-cerebral-angiogram-in-the-workup-of-central-nervous-system-vasculitis
#9
James Monroe Wright, Berje Haroutuon Shammassian, Jeffrey Tait Nelson, Christina Huang Wright
No abstract text is available yet for this article.
August 1, 2016: Neurosurgery
https://www.readbyqxmd.com/read/28149658/do-clinical-findings-of-beh%C3%A3-et-s-disease-vary-by-gender-a-single-center-experience-from-329-patients
#10
Döndü Üsküdar Cansu, Timuçin Kaşifoğlu, Cengiz Korkmaz
OBJECTIVE: Behçet's disease (BD) is a systemic vasculitis with a significantly varying clinical course following relapses and remissions, which may involve a number of organs such as the skin, joints, lungs, and blood vessels as well as systems such as the central nervous system and gastrointestinal system. Its prognosis is known to be worse in males. There are several studies in the literature on the clinical features and gender distribution of BD. The aim of the present study was to determine the clinical characteristics of BD and the presence of a relation with gender and to investigate the correlation of our results with the current literature...
December 2016: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28087351/central-nervous-system-vasculitis-in-adults-an-update
#11
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/28079834/intestinal-brucellosis-associated-with-celiac-artery-and-superior-mesenteric-artery-stenosis-and-with-ileum-mucosa-and-submucosa-thickening-a-case-report
#12
Miaoqian Wang, Qingli Zhu, Qian Yang, Wenbo Li, Xinning Wang, Wei Liu, Baotong Zhou, Zhenghong Li, Hong Yang
RATIONALE: Brucellosis is a multisystem infection found worldwide that has a broad range of characteristics, which range from acute fever and hepatomegaly to chronic infections that most commonly affect the central nervous system, cardiovascular system, or skeletal system. Gastrointestinal and splanchnic artery involvements in brucellosis are relatively uncommon. PATIENT CONCERNS: We report a case of brucellosis in an adolescent presenting as intermittent abdominal pain, diarrhea, and fever, with intestinal tract involvement...
January 2017: Medicine (Baltimore)
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/28062430/rare-and-unusual-case-of-polyarteritis-nodosa-involving-the-gastrointestinal-tract-leading-to-bowel-gangrene
#14
Arvind Vashdev Jagwani, Nik Qisti Fathi, Ruhi Fadzlyana Jailani, Andee Dzulkarnaen Zakaria
Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis preferentially targeting medium-sized arteries and not related with glomerulonephritis or small vessel involvement. Clinical manifestations of PAN are multisystem. The gastrointestinal, renal, cardiac, musculoskeletal, skin and central nervous systems may be involved. The aetiology remains unknown, and the ensuing vasculitis may lead to aneurysm formation and thrombosis in any organs of the body with resultant ischaemia. PAN of the intestines is a relatively common manifestation of this disease but rarely causes bowel ischaemia resulting in necrosis...
January 6, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28034820/mycophenolate-mofetil-as-induction-and-long-term-maintaining-treatment-in-childhood-primary-angiitis-of-the-central-nervous-system
#15
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)...
December 26, 2016: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28031844/refractory-rheumatoid-vasculitis
#16
Ashok Kumar, Anshul Goel, Mehul Lapsiwala, Suman Singhal
Systemic rheumatoid vasculitis (SRV) can develop in rheumatoid arthritis of long duration and high disease activity. It most commonly manifests as cutaneous vasculitis and mononeuritis multiplex. This can involve any organ of the body and carries very high mortality. We report a case of a young male who had rheumatoid arthritis for the past 15 years and became refractory to standard drugs and anti-TNF agents. He subsequently developed SRV, which started as mononeuritis multiplex. Disease progressed to result in gangrene of hands and feet despite receiving intravenous cyclophosphamide...
November 2016: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28024309/stroke-as-initial-manifestation-of-adenosine-deaminase-2-deficiency
#17
Miriam Elbracht, Michael Mull, Norbert Wagner, Christiane Kuhl, Angela Abicht, Ingo Kurth, Klaus Tenbrock, Martin Häusler
Deficiency of adenosine deaminase 2 (ADA2) due to homozygous or compound heterozygous mutations in the cat eye syndrome chromosome region, candidate 1 (CECR1) gene causes an autoimmune phenotype with systemic vasculitis affecting the skin, inner organs, and the central nervous system. Typically, stroke has been reported to follow systemic inflammatory disease and predominantly affects posterior and central brain areas. Here, we describe one of the rare patients in whom acute mesencephalic stroke preceded systemic inflammation and presented as initial clinical symptom...
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28004435/biopsy-proven-case-of-epstein-barr-virus-ebv-associated-vasculitis-of-the-central-nervous-system
#18
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/27998307/neuro-behcet-disease-presenting-as-a-solitary-cerebellar-hemorrhagic-lesion-a-case-report-and-review-of-the-literature
#19
Minju Yeo, Hye-Lim Lee, Minju Cha, Ji Seon Kim, Ho-Seong Han, Sung-Hyun Lee, Sang-Soo Lee, Dong-Ick Shin
BACKGROUND: Behcet's disease is a heterogeneous, multisystem, inflammatory disorder of unknown etiology. The classic triad of oral and genital ulcerations in conjunction with uveitis was originally described by the Turkish dermatologist Hulusi Behcet in 1937, but associated symptoms of the cardiovascular, central nervous, pulmonary, and gastrointestinal systems were later identified. In fact, Behcet's disease with neurological involvement (neuro-Behcet's disease) is not uncommon. Patients with neuro-Behcet's disease typically exhibit a diverse array of symptoms, most commonly in the brainstem and diencephalic regions...
December 20, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27987517/-cerebral-infarction-in-a-patient-with-primary-sjogren-s-syndrome-a-case-report-and-literature-review
#20
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
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