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primary angiitis of the central nervous system

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https://www.readbyqxmd.com/read/28456915/reversible-cerebral-vasoconstriction-syndrome-recognition-and-treatment
#1
REVIEW
Cecilia Cappelen-Smith, Zeljka Calic, Dennis Cordato
Reversible cerebral vasoconstriction syndrome (RCVS) is a rare but increasingly recognized disorder with over 500 cases published in the literature. The condition is characterized by recurrent severe thunderclap headaches with or without other neurological symptoms and diffuse segmental narrowing of the cerebral arteries which is reversible within 3 months. RCVS may occur spontaneously but in over 50% of cases, it is associated with various other conditions, including vasoactive medications or illicit drugs and the post-partum state...
June 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28419338/primary-angiitis-of-the-central-nervous-system-a-territory-about-to-be-discovered
#2
Thomas Daikeler, Alan Tyndall
No abstract text is available yet for this article.
April 16, 2017: Rheumatology
https://www.readbyqxmd.com/read/28355722/-a-clinical-analysis-of-5-patients-with-infratentorial-primary-angiitis-of-central-nervous-system
#3
L J Peng, H R Qian, L L Mao, D Y Xia, X K Qi
Objective: To explore the clinical characteristics of infratentorial primary angiitis in central nervous system(PACNS). Methods: A total of 5 cases diagnosed as infratentorial PACNS in the neurology department of Navy General Hospital of PLA in 2015 were enrolled in the study. The clinical, imaging and pathological data were collected and analyzed. Results: All the 5 cases were male with the median onset age of thirty-four. Five cases presented with dizziness, two with headache, three with walking unstable, two with facial numbness and one with dysarthria...
April 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28340158/maintenance-therapy-is-associated-with-better-long-term-outcomes-in-adult-patients-with-primary-angiitis-of-the-central-nervous-system
#4
Hubert de Boysson, Jean-Jacques Parienti, Caroline Arquizan, Grégoire Boulouis, Nicolas Gaillard, Alexis Régent, Antoine Néel, Olivier Detante, Emanuel Touzé, Achille Aouba, Boris Bienvenu, Loïc Guillevin, Olivier Naggara, Mathieu Zuber, Christian Pagnoux
Objective.: We aimed to analyse the effect of maintenance therapy after induction on the outcomes of adult patients with primary angiitis of the CNS (PACNS). Methods.: We analysed long-term outcomes (relapse, survival and functional status) of patients enrolled in the French multicentre PACNS cohort who achieved remission after induction treatment and with ⩾12 months' follow-up, according to whether or not they received maintenance therapy. Good outcome was defined as relapse-free survival and good functional status (modified Rankin scale ⩽ 2) at last follow-up...
March 17, 2017: 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
#5
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/28248859/seizure-syndrome-as-a-first-manifestation-of-solitary-tumor-like-mass-lesion-of-pacns-two-case-reports
#6
De-Sheng Zhu, Xiao-Li Yang, Hui-Hui Lv, Chen-Guang Bai, Pang-Pan Yang, Ze-Zhi Li, Yong Hao, Ying Zhang, Yang-Tai Guan
RATIONALE: Primary angiitis of the central nervous system (PACNS) is an inflammatory disease involving cerebrovascular and parenchymal, and solitary tumor-like mass lesion of PACNS (TLML-PACNS) is frequently misdiagnosed as neoplastic or other inflammatory diseases. However, seizure syndrome as a first manifestation of TLML-PACNS has rarely reported before. PATIENT CONCERNS: Here, we report 2 cases of seizure syndrome, which was the first sign that presented prior to the diagnosis of TLML-PACNS by brain biopsy...
March 2017: Medicine (Baltimore)
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/28034820/mycophenolate-mofetil-as-induction-and-long-term-maintaining-treatment-in-childhood-primary-angiitis-of-the-central-nervous-system
#8
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/27940585/adult-primary-angiitis-of-the-central-nervous-system-isolated-small-vessel-vasculitis-represents-distinct-disease-pattern
#9
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/27864289/immunotherapy-for-arterial-ischaemic-stroke-in-childhood-a-systematic-review
#10
REVIEW
Hannah B Edwards, Andrew A Mallick, Finbar J K O'Callaghan
BACKGROUND: There is little evidence about either prevention or treatment of childhood arterial ischaemic stroke (AIS). However, drugs that regulate the immune and inflammatory response could theoretically prevent occurrence or recurrence of AIS. Additionally, as an acute treatment, they may limit the neurological damage caused by AIS. Here, we systematically review the evidence on the use of immunotherapy in childhood AIS. DESIGN: A systematic review of publications in databases Embase and Medline from inception...
May 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27618323/white-matter-diseases-with-radiologic-pathologic-correlation
#11
Nicolae Sarbu, Robert Y Shih, Robert V Jones, Iren Horkayne-Szakaly, Laura Oleaga, James G Smirniotopoulos
White matter diseases include a wide spectrum of disorders that have in common impairment of normal myelination, either by secondary destruction of previously myelinated structures (demyelinating processes) or by primary abnormalities of myelin formation (dysmyelinating processes). The pathogenesis of many white matter diseases remains poorly understood. Demyelinating disorders are the object of this review and will be further divided into autoimmune, infectious, vascular, and toxic-metabolic processes. Autoimmune processes include multiple sclerosis and related diseases: tumefactive demyelinating lesions, Balo concentric sclerosis, Marburg and Schilder variants, neuromyelitis optica (Devic disease), acute disseminated encephalomyelitis, and acute hemorrhagic leukoencephalopathy (Hurst disease)...
September 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/27570401/primary-cns-vasculitis-presenting-as-intraventricular-bleeding
#12
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
https://www.readbyqxmd.com/read/27554617/mri-and-clinicopathological-correlation-of-childhood-primary-central-nervous-system-angiitis
#13
N Wilson, D Pohl, J Michaud, A Doja, E Miller
AIM: To review the long-term clinical and imaging follow-up of eight children with histopathologically proven childhood primary central nervous system angiitis (cPACNS). MATERIALS AND METHODS: Eight children (5-17 years, five female and three male patients) with biopsy-proven cPACNS vasculitis were reviewed retrospectively. All children were followed at the Children's Hospital of Eastern Ontario. Magnetic resonance imaging (MRI) images of the brain parenchyma and vessel pattern of the arteries of the circle of Willis were reviewed at baseline and follow-up(s)...
November 2016: Clinical Radiology
https://www.readbyqxmd.com/read/27476630/intracerebral-hemorrhage-with-a-favorable-outcome-in-a-patient-with-childhood-primary-angiitis-of-the-central-nervous-system
#14
Marco Felipe Silva, Ana Raquel Feitosa, José A Paz, Nádia Emi Aikawa, Clovis A Silva
Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation...
July 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27470990/tumor-like-presentation-of-primary-angiitis-of-the-central-nervous-system
#15
Hubert de Boysson, Grégoire Boulouis, Nelly Dequatre, Sophie Godard, Antoine Néel, Caroline Arquizan, Olivier Detante, Coralie Bloch-Queyrat, Mathieu Zuber, Emmanuel Touzé, Boris Bienvenu, Achille Aouba, Loïc Guillevin, Olivier Naggara, Christian Pagnoux
BACKGROUND AND PURPOSE: We aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system. METHODS: We retrospectively analyzed 10 patients enrolled in the French primary angiitis of the central nervous system cohort, who initially presented tumor-like brain lesions and compared them with other patients within the cohort. RESULTS: The 10 patients with tumor-like presentation in the cohort were younger and had more seizures at diagnosis than the other 75 patients (median of 37 [30-48] years versus 46 [18-79] years; P=0...
September 2016: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27469076/vascular-imaging-outcomes-of-childhood-primary-angiitis-of-the-central-nervous-system
#16
Jorina Elbers, Derek Armstrong, Ivanna Yau, Susanne Benseler
BACKGROUND: Inflammation affecting cerebral blood vessels is a common cause of stroke in children. Arterial abnormalities on vascular imaging are an important risk factor for stroke recurrence. We aimed to describe the vascular imaging outcomes in children with primary angiitis of the central nervous system after 12 months and identify factors associated with vascular progression and stroke recurrence. METHODS: We retrospectively analyzed clinical and neuroimaging data from the BrainWorks Registry of children with large-vessel primary angiitis of the central nervous system...
October 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/27432677/other-noninfectious-inflammatory-disorders
#17
REVIEW
Álex Rovira, Cristina Auger, Antoni Rovira
Idiopathic inflammatory-demyelinating diseases (IIDDs) represent a broad spectrum of central nervous system (CNS) disorders, including monophasic, multiphasic, and progressive disorders that range from highly localized forms to multifocal or diffuse variants. In addition to the classic multiple sclerosis (MS) phenotypes, several MS variants have been described, which can be differentiated on the basis of severity, clinical course, and lesion distribution. Other forms of IIDD are now recognized as distinct entities and not MS variants, such as acute disseminated encephalomyelitis, and neuromyelitis optica spectrum disorders...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27354225/diagnostic-yield-and-safety-of-brain-biopsy-for-suspected-primary-central-nervous-system-angiitis
#18
Jose Torres, Caitlin Loomis, Brett Cucchiara, Michelle Smith, Steven Messé
BACKGROUND AND PURPOSE: The utility and safety of brain biopsy for suspected primary angiitis of the central nervous system (PACNS) are uncertain. Factors predictive of a positive biopsy have not been well described. Our aim was to evaluate the diagnostic yield and safety of brain biopsy in suspected PACNS and determine whether any prebiopsy variables are associated with a positive biopsy. METHODS: This is a retrospective study of consecutive patients who underwent diagnostic brain biopsy for PACNS at a single institution...
August 2016: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27123289/a-rare-case-of-tumor-mimicking-primary-angiitis-of-the-central-nervous-system
#19
L I Sun, Lijun Zhu, Teng Zhao, Dayan Wang, Dihui Ma, Rensheng Zhang, Shaokuan Fang
Primary angiitis of the central nervous system (PACNS) is a rare, but severe vascular disease. The present study reports the case of a 42-year-old male who developed PACNS. Magnetic resonance imaging (MRI) scans initially led to a misleading diagnosis of malignant glioma, and surgery was performed. The mass was resected, and a pathological examination confirmed a cerebral vasculitis. Single therapy with high doses of steroid did not improve the patient's condition, while a subsequent lesion appeared on the opposite side one year later...
May 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27112683/central-nervous-system-vasculitis-in-adults-and-children
#20
REVIEW
Marinka Twilt, Susanne M Benseler
Primary angiitis of the central nervous system (PACNS) is an inflammatory brain disease targeting the cerebral blood vessels, leading to a wide spectrum of signs and symptoms, including neurologic deficits, cognitive dysfunction, and psychiatric symptoms. The inflammation could be reversible if diagnosed and treated early. The diagnosis requires the careful consideration and rapid evaluation of systemic underlying conditions and disease mimics. The differential diagnosis is distinctly different for angiography-positive and -negative PACNS subtypes and differs depending on age, so there is childhood PACNS or adult PACNS...
2016: Handbook of Clinical Neurology
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