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Cerebral 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/28893009/intracranial-aneurysms-involving-circle-of-willis-in-a-child-with-human-immunodeficiency-virus-associated-vasculitis-a-rare-case-report
#2
Amol Madanlal Lahoti, Abhijit Kishor Taori, Avinash Parashuram Dhok, Jitesh Subhash Rawat, Nihar Umakant Chandak
Intracranial Arterial Aneurysms (IAAs) are relatively rare in paediatric population and they account for at least 10%-15% of haemorrhagic strokes which occur during the first two decades of life. Here we present a unique and unusual case of Human Immunodeficiency Virus (HIV) infected child who presented with intracranial aneurysms with formation of collaterals and vasculopathy, demonstrating low viral count despite receiving adequate antiretroviral treatment. Intracranial vascular involvement, their complications and its incidence in these patients may become increasingly common as the management of human immunodeficiency virus infection continues to improve and afflicted patients survive for longer periods because of advancement in the antiretroviral treatment...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28864038/radiologically-isolated-cerebral-amyloid-angiopathy-related-inflammation
#3
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/28809763/persistent-placoid-maculopathy-complicated-by-cerebral-vasculitis
#4
Eva Lenassi, Maja Kojovic, Polona Jaki Mekjavić, Sasa Sega, Natasa Vidovič Valentinčič
Persistent placoid maculopathy (PPM) is a bilateral inflammatory chorioretinopathy characterized by long-standing plaque-like macular lesions. No systemic manifestations have been reported to date. We describe a case of PPM complicated by cerebral vasculitis, suggesting that neurological symptoms, including headache, should be enquired about in all PPM subjects.
September 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
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
#5
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/28767023/-cerebral-vasculitis-a-diagnostic-labyrinth
#6
R C van Houwelingen, C J M Frijns, S T Toering, P J van Laar, J F Meilof
- A diagnosis of cerebral vasculitis is frequently considered in patients with new or progressive neurological symptoms for which there is no other explanation.- A clinician considering a diagnosis of cerebral vasculitis should be well aware of alternative diagnoses, since these are generally more common.- Several consecutive examinations are required for diagnosing cerebral vasculitis, because there is no diagnostic procedure that is highly sensitive as well as highly specific.- The added value of the different procedures may depend on the type of blood vessels involved...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28764590/vasculitis-causing-massive-infarction-a-rare-presentation-of-primary-cerebral-phaeohyphomycosis
#7
Rajalakshmi Poyuran, B H Srinivas, Bhawana A Badhe
No abstract text is available yet for this article.
January 1, 2017: Tropical Doctor
https://www.readbyqxmd.com/read/28743241/complete-ophthalmoplegia-complete-ptosis-and-dilated-pupil-due-to-internal-carotid-artery-dissection-as-the-first-manifestation-of-takayasu-arteritis
#8
H M M T B Herath, S P Pahalagamage, D Withana, Sunethra Senanayake
BACKGROUND: Takayasu arteritis is a rare, chronic large vessel vasculitis involving the aorta and its primary branches. As the disease progresses, the active inflammation of large vessels leads to dilation, narrowing and occlusion of the arteries. Arterial dissection is due to separation of the layers of the arterial wall resulting in a false lumen, where blood seeps into the vessel wall. Neurological sequelae of intracranial arterial dissection results from cerebral ischemia due to thromboembolism and hypo perfusion...
July 25, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28739362/stroke-in-ehlers-danlos-syndrome-kyphoscoliotic-type-dissection-or-vasculitis
#9
Annegret Quade, Martin Wiesmann, Joachim Weis, Ingo Kurth, Houman Jalaie, Marianne Rohrbach, Martin Häusler
BACKGROUND: Patients with the kyphoscoliotic type of Ehlers-Danlos syndrome have an increased risk of vascular complications such as aortic dissection and perforation. Cerebral ischemia has only rarely been documented. PATIENT DESCRIPTION: This 13-year-old girl with the kyphoscoliotic type of Ehlers-Danlos syndrome experienced a large right middle cerebral artery distribution infarction. Full intravenous heparinization was started in response to presumed arterial dissection...
September 2017: Pediatric Neurology
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
#10
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/28724595/hypertensive-encephalopathy-mimicking-cerebral-vasculitis-with-pontine-oedema-cerebellar-white-matter-lesions-and-multiple-cerebral-infarctions
#11
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/28680302/eosinophilic-granulomatosis-with-polyangiitis-presented-as-acute-polyneuropathy-and-cerebral-vasculitis
#12
Il-Han Yoo, Sang Tae Choi, Seong-Ho Choi, Jeong-Min Kim, Suk-Won Ahn
Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs...
June 2017: Experimental Neurobiology
https://www.readbyqxmd.com/read/28642817/cerebral-vasculitis-mimicking-intracranial-metastatic-progression-of-lung-cancer-during-pd-1-blockade
#13
Heinz Läubli, Jürgen Hench, Michal Stanczak, Ingmar Heijnen, Alexandros Papachristofilou, Stephan Frank, Alfred Zippelius, Frank Stenner-Liewen
BACKGROUND: Stimulation of the immune system by targeting the PD-1/PD-L1 pathway can result in activation of anti-tumor immunity. Besides its clinical benefit immune checkpoint therapy leads to significant immune-related adverse events (irAEs). Some rare irAEs are not well described yet but are critical in patient management. CASE PRESENTATION: Here, we describe a case of autoimmune cerebral vasculitis/encephalitis after PD-1 inhibitor treatment for metastatic adenocarcinoma of the lung...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28631704/-beh%C3%A3-et-s-disease-intracardiac-thrombosis-a-description-of-two-cases-and-a-review-of-literature
#14
Z S Alekberova, P S Ovcharov, T A Lisitsyna, A V Volkov, T V Popkova
Behçet's disease (BD) is systemic vasculitis of unknown etiology, which is more common in the countries located along the Great Silk Road. The disease is diagnosed if a patient has 4 key diagnostic signs: aphthous stomatitis, genital sores, and eye and skin lesions. Vascular diseases referred to as minor criteria for BD are characterized by the formation of aneurysms and thrombosis, predominantly in the venous bed. In venous disorders, a blood clot can form in any vessel, including caval, cerebral, pulmonary, and other veins...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28614161/multiple-cerebral-infarcts-in-a-young-patient-associated-with-marijuana-use
#15
Leila Costa Volpon, Camila Lacerda Muniz de Melo Sousa, Silvia Keiko Kavaguti Moreira, Sara Reis Teixeira, Ana Paula de Carvalho Panzeri Carlotti
: Cerebrovascular events associated with marijuana use have been reported previously. This association is plausible, but not well-established yet. A 14-year-old girl, long-term heavy cannabis user, presented with generalized tonic-clonic seizures and decreased level of consciousness a few hours after smoking cannabis. Brain magnetic resonance imaging showed multiple areas of acute, subacute and chronic ischemic lesions in the left frontal lobe, basal ganglia, and corpus callosum. History of other illicit drug use and other known causes of stroke were ruled out...
June 13, 2017: Journal of Addiction Medicine
https://www.readbyqxmd.com/read/28608454/accelerated-atherosclerosis-in-anca-associated-vasculitis
#16
I González-Suárez, J J Ríos-Blanco, J Arpa
OBJECTIVES: Cardiovascular disease, including myocardial infarction and stroke, is a major cause of mortality in ANCA-associated vasculitis (AAV). Although AAV affects small vessels, an accelerated atherosclerosis not explained by traditional cardiovascular risk factors (CVRF) has been demonstrated. We aimed to investigate the association of atherosclerosis measured by carotid intima-media thickness (CIMT) and cerebral small vessel disease in AAV-patients. MATERIALS & METHODS: Twenty-three AAV-patients in complete remission were recruited...
June 12, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28578629/chaetomiaceae-fungi-novel-pathogens-of-equine-neurotropic-phaeohyphomycosis
#17
Quinci Plumlee, Courtney Meason-Smith, Alexandra Dieterly, Gabriel Gomez, Brian F Porter, Aline Rodrigues Hoffmann
Many previously unrecognized fungi are emerging as potential pathogens. One such group is dematiaceous fungi of the Chaetomiaceae family (phylum Ascomycota, class Sordariomycetes). These fungi are rare causes of opportunistic, neurotropic phaeohyphomycosis in humans but are not known to cause similar infections in animals. The aims of this study were to investigate equine hyphal mycotic encephalitis, characterize key histopathologic features, and classify causative organisms with molecular diagnostic techniques...
September 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28566939/variation-of-a-persistent-primitive-hypoglossal-artery-ppha-as-incidental-finding-in-the-diagnostic-clarification-of-cerebral-vasculopathy-associated-with-intracranial-vasculitis
#18
S Hopf-Jensen, L Marques, M Preiß, W Börm, S Müller-Hülsbeck
We present a very rare variation of a persistent primitive hypoglossal artery (PPHA) arising from the internal carotid artery, detected during a diagnostic angiography. A 50-year-old female patient was admitted with an atypical intracranial hematoma in the left frontal lobe. Catheter angiography revealed intracranial vasculopathy with segmental stenoses, a small aneurysm of the right internal carotid artery bifurcation and a "string of beads" appearance of the left carotid artery, consistent with fibromuscular disease...
June 2017: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
https://www.readbyqxmd.com/read/28527263/-racemose-neurocysticercosis-neuroimaging-guides-the-diagnosis
#19
Carlos Hugo Zapata, Sergio Alberto Vargas, Carlos Santiago Uribe
Neurocysticercosis is the leading cause of parasitosis of the central nervous system and acquired epilepsy in developing countries. The clinical manifestations of neurocysticercosis, especially its racemose variant, are pleomorphic and unspecific, characteristics that hinder the diagnosis and make it a challenge for the clinician.The objective of this report was to describe two cases of racemose neurocysticercosis in which neuroimaging led to the definitive diagnosis. The first case involved a patient with persistent headache and focal neurological signs...
April 1, 2017: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/28524121/-the-use-of-high-flow-extracranial-intracranial-artery-bypass-in-pathology-of-the-cerebral-and-brachiocephalic-arteries-technical-features-and-surgical-outcomes
#20
A V Dubovoy, K S Ovsyannikov, V E Guzhin, A V Cherepanov, D M Galaktionov, A M Perfil'ev, A O Sosnov
BACKGROUND: Poor outcomes of surgical treatment for complex cerebral aneurysms due to the development of cerebral ischemia were the cause to use cerebral revascularization surgery for this pathology. OBJECTIVE: the study objective was to master a high-flow extracranial-intracranial (EC-IC) artery bypass technique and evaluate its application in surgical treatment of complex and giant cerebral aneurysms as well as complex lesions of the brachiocephalic arteries. MATERIAL AND METHODS: Fifty two patients underwent high-flow IC-EC bypass surgery; of these, 34 patients had complex cerebral aneurysms, and 18 patients had complex stenotic occlusive lesions of the brachiocephalic arteries...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
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