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primary angiitis of the central nervous system

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https://www.readbyqxmd.com/read/27864289/immunotherapy-for-arterial-ischaemic-stroke-in-childhood-a-systematic-review
#1
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...
November 18, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27618323/white-matter-diseases-with-radiologic-pathologic-correlation
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/27043703/reversible-cerebral-vasoconstriction-syndromes-and-primary-angiitis-of-the-central-nervous-system-clinical-imaging-and-angiographic-comparison
#12
Aneesh B Singhal, Mehmet A Topcuoglu, Joshua W Fok, Oguzhan Kursun, Raul G Nogueira, Matthew P Frosch, Verne S Caviness
Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. However, prompt and accurate diagnosis remains challenging. Here we compared the features of 159 RCVS to 47 PACNS patients and developed criteria for prompt bedside diagnosis. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders...
June 2016: Annals of Neurology
https://www.readbyqxmd.com/read/26951336/coexisting-vascular-lesions-in-reversible-cerebral-vasoconstriction-syndrome
#13
M Akif Topcuoglu, Oguzhan Kursun, Aneesh B Singhal
BACKGROUND: The pathophysiology of reversible cerebral vasoconstriction syndrome (RCVS) is not known. Published cases have documented coexisting cervical artery dissection and unruptured aneurysms, raising the possibility that ultrastructural vessel wall abnormalities underlie the development of vascular lesions as well as RCVS. METHODS: In this retrospective study we compared the frequency of neurovascular abnormalities in 158 consecutive RCVS patients, 44 patients with primary angiitis of the central nervous system (PACNS, positive controls), and 177 non-stroke patients with acute neurological symptoms (non-arteriopathy controls)...
March 7, 2016: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/26852378/vessel-wall-enhancement-in-the-diagnosis-and-management-of-primary-angiitis-of-the-central-nervous-system-in-children
#14
Koyo Ohno, Yoshiaki Saito, Hirofumi Kurata, Yusuke Saiki, Hiroko Ohtahara, Hiroki Yoshioka, Eijiro Yamashita, Shinya Fujii, Yoshihiro Maegaki
We describe two cases of primary angiitis of the central nervous system in children (cPACNS) diagnosed by vessel wall contrast enhancement on magnetic resonance imaging (MRI). Both patients developed acute cerebral infarction after fever and malaise. In patient 1, a 7-month-old boy, MRI revealed extensive cerebral infarction in the right middle cerebral artery (MCA) area and stenosis at the M1 portion of the right MCA. Oral glucocorticoid therapy was initiated. Vessel wall enhancement was ameliorated 3months after onset, and stenosis was mostly restored...
August 2016: Brain & Development
https://www.readbyqxmd.com/read/26837371/primary-angiitis-of-the-central-nervous-system-avoiding-misdiagnosis-and-missed-diagnosis-of-a-rare-disease
#15
REVIEW
Shamik Bhattacharyya, Aaron L Berkowitz
Primary angiitis of the central nervous system (PACNS) is an extremely rare condition, defined as a vasculitis limited to the CNS with no identifiable cause. Its presentation is non-specific and includes headache, cognitive dysfunction and focal neurological signs. Laboratory studies, neuroimaging and angiography are neither sufficiently sensitive nor specific for diagnosis; a definitive diagnosis requires brain biopsy. As a result, PACNS is commonly misdiagnosed. Here, we review its clinical, laboratory and radiological features, and focus on avoiding common diagnostic pitfalls...
June 2016: Practical Neurology
https://www.readbyqxmd.com/read/26778046/ten-year-clinical-experience-with-stroke-and-cerebral-vasculitis
#16
Peter A Kempster, Catriona A McLean, Thanh G Phan
Angiitis of the central nervous system (CNS) is difficult to diagnose but potentially fatal. When stroke occurs in a younger individual or is associated with multiple infarcts on imaging, clinicians must decide how far to pursue a possible diagnosis of vasculitis. The aim of this study is to establish the prevalence of primary and secondary cerebral angiitis among patients presenting with stroke. Hospital attendances over a 10year period were surveyed by searching for diagnostic codes and key words specific for cerebral vasculitis/angiitis...
May 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/26773697/frontal-lobe-dementia-syndrome-as-a-first-manifestation-of-primary-angiitis-of-the-central-nervous-system-pacns
#17
Marlene Bönstrup, Katja Ott, Markus Glatzel, Tim Magnus
This case presents a clinical course of a frontal lobe dysexecutive syndrome with dementia caused by a primary angiitis of the central nervous system (PACNS) of exclusively very small vessels. An isolated frontal lobe dementia syndrome as a primary manifestation of PACNS highlights the diverse clinical manifestations of the disease. The patient presented with a progressive cognitive decline with loss of memory, disinhibited behavior, inappropriate affect and frontal release signs. The diagnostic workup essentially revealed a lymphocytic pleocytosis in the cerebrospinal fluid and a generalized cortical atrophy without any vascular abnormalities...
February 2016: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/26571763/-case-report-juvenile-cerebral-infarction-due-to-primary-angiitis-of-the-central-nervous-system
#18
Makoto Terada, Kiyotaka Nakamagoe, Akira Tamaoka
No abstract text is available yet for this article.
June 10, 2015: Nihon Naika Gakkai Zasshi. the Journal of the Japanese Society of Internal Medicine
https://www.readbyqxmd.com/read/26198847/central-nervous-system-granulomastous-phlebitis-with-limited-extracranial-involvement-of-the-heart-and-lungs-an-autopsy-case
#19
Jernej Mlakar, Jerneja Videčnik Zorman, Mojca Matičič, Matej Vrabec, Armin Alibegović, Mara Popović
Primary angiitis of the central nervous system is a rare condition, usually with an insidious onset. There is a wide variety of histological types (granulomatous, lymphocytic or necrotizing vasculitis) and types of vessel involved (arteries, veins or both). Most cases are idiopathic. We describe a first case of idiopathic granulomatous central nervous system phlebitis with additional limited involvement of the heart and lung, exclusively affecting small and medium sized veins in a 22-year-old woman, presenting as a sub acute headache...
February 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/26185185/inflammatory-cerebral-amyloid-angiopathy-amyloid-%C3%AE-related-angiitis-and-primary-angiitis-of-the-central-nervous-system-similarities-and-differences
#20
Aimen Moussaddy, Ariel Levy, Daniel Strbian, Sophia Sundararajan, France Berthelet, Sylvain Lanthier
No abstract text is available yet for this article.
September 2015: Stroke; a Journal of Cerebral Circulation
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