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central nervous system vasculitis

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https://www.readbyqxmd.com/read/28087351/central-nervous-system-vasculitis-in-adults-an-update
#1
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 include 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 data large-vessel PCNSV present worse prognosis when compared to small-vessel PCNSV...
January 10, 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
#2
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
#3
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...
January 10, 2017: Pathology International
https://www.readbyqxmd.com/read/28062430/rare-and-unusual-case-of-polyarteritis-nodosa-involving-the-gastrointestinal-tract-leading-to-bowel-gangrene
#4
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
#5
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
#6
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
#7
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...
December 26, 2016: Neuropediatrics
https://www.readbyqxmd.com/read/28004435/biopsy-proven-case-of-epstein-barr-virus-ebv-associated-vasculitis-of-the-central-nervous-system
#8
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
#9
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
#10
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/27955815/salmonella-meningitis-associated-with-monocyte-infiltration-in-mice
#11
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/27940591/granulomatosis-with-polyangiitis-according-to-geographic-origin-and-ethnicity-clinical-biological-presentation-and-outcome-in-a-french-population
#12
Benjamin Terrier, Agnès Dechartres, Christophe Deligny, Pascal Godmer, Pierre Charles, Gilles Hayem, Bertrand Dunogué, Michel de Bandt, Pascal Cohen, Xavier Puéchal, Claire Le Jeunne, Serge Arfi, Luc Mouthon, Loïc Guillevin
OBJECTIVES: Granulomatosis with polyangiitis (GPA) mainly affects white Europeans, but rarely GPA may also affect non-Europeans. This study aimed to describe GPA clinical-biological presentation and outcome in black sub-Saharan Africans and Afro-Caribbeans and in North Africans. METHODS: Among 914 GPA patients included in the French Vasculitis Study Group database, geographic origin and ethnicity were known for 760. Clinical-biological presentations and outcomes of white Europeans vs black sub-Saharans and Afro-Caribbeans and vs North Africans were analysed...
December 10, 2016: Rheumatology
https://www.readbyqxmd.com/read/27940585/adult-primary-angiitis-of-the-central-nervous-system-isolated-small-vessel-vasculitis-represents-distinct-disease-pattern
#13
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/27919794/diagnostic-utility-of-contrast-enhanced-3d-t1-weighted-imaging-in-acute-cerebral-infarction-associated-with-graves-disease
#14
Yasufumi Gon, Manabu Sakaguchi, Naoki Oyama, Hideki Mochizuki
Graves disease is rarely complicated with cerebrovascular steno-occlusive diseases. Previous studies have suggested several hypotheses for this occurrence, including excess thyroid hormone, which stimulates the sympathetic nervous system, which in turn causes an abnormal hemodynamic response with consequent atherosclerotic changes, and antithyroid antibodies cause local vascular inflammation in patients with Graves disease. However, radiological findings of vasculitis in patients with Graves disease and cerebral infarction remain less known...
December 2, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/27917269/ecstasy-mdma-and-its-effects-on-kidneys-and-their-treatment-a-review
#15
REVIEW
Feyza Bora, Fatih Yılmaz, Taner Bora
Ecstasy (MDMA; 3,4-methylenedioxymethylamphetamine) is an illicit drug that has been increasingly abused by young people. Its effects include euphoria, enhanced sociability and heightened mental awareness. These come about via the increase of serotonin in both the central nervous system and the sympathetic nervous system. Despite the drug's prevalent abuse, serious or adverse effects are rare. Due to personal pharmacokinetics, effects from the same dosage vary according to the individual. Fatal instances may include acute hyponatremia, hyperthermia (>42 °C), disseminated intravascular coagulation (DIC) resulting from hyperthermia affecting the kidneys, and non-traumatic rhabdomyolysis...
November 2016: Iranian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/27894445/usual-and-unusual-manifestations-of-systemic-and-central-nervous-system-vasculitis
#16
REVIEW
James J Nocton
The idiopathic vasculitides are a group of inflammatory and immune-mediated conditions associated with inflammation of blood vessels. They affect multiple organ and body systems, and vary in their clinical manifestations, severity, prognosis, and pathology. They frequently present a diagnostic challenge for clinicians because of their complexity, overlapping features, and similar findings to other noninflammatory, genetic, or infectious conditions. This article summarizes some of the common pediatric vasculitides, emphasizing both the characteristic and unusual clinical manifestations of these diseases...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27884882/lymphomatosis-cerebri-diagnostic-challenges-and-review-of-the-literature
#17
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/27870601/t-cell-immunity-to-varicella-zoster-virus-in-the-setting-of-advanced-hiv-and-multiple-varicella-zoster-virus-recurrences
#18
Rachel A Bender Ignacio, Meena S Ramchandani, Kerry J Laing, Christine M Johnston, David M Koelle
A woman presented with at least four manifestations of varicella-zoster virus (VZV) infection, including central nervous system vasculitis, during her first 2 years of HIV infection. We evaluated her CD4 T cell responses to VZV given the infrequency with which multiple recurrences of VZV occurred, especially following immune reconstitution on antiretroviral therapy.
January 2017: Viral Immunology
https://www.readbyqxmd.com/read/27818812/cns-vasculitis-associated-with-waldenstr%C3%A3-m-macroglobulinemia
#19
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
https://www.readbyqxmd.com/read/27812073/eosinophilic-granulomatosis-with-polyangiitis-presenting-with-multiple-intracerebral-haemorrhages-a-case-report
#20
Zafar Ullah, Zain Ul Abideen, Raja Farhat Shoaib, Arsalan Ahmad, Qamar Zaman, Rashid Nazir
Eosinophilic granulomatosis with polyangiitis also known as Churgg-Strauss syndrome is a systemic multi system vasculitis. Neurological involvement is mostly in the form of peripheral nervous system disease. Central nervous system involvement is relatively uncommon with most cases being secondary to ischaemic infarctions. Intra cerebral haemorrhage is rare and is usually in the form of solitary haemorrhagic lesions. Multiple intra cerebral haemorrhages are exceptionally rare with only one case documented in medical literature...
November 2016: JPMA. the Journal of the Pakistan Medical Association
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