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primary angiitis of the central nervous system

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https://www.readbyqxmd.com/read/29062691/new-onset-refractory-status-epilepticus-due-to-primary-angiitis-of-the-central-nervous-system
#1
Rawan K Matar, Bader Alshamsan, Saleh Alsaleh, Hindi Alhindi, Khalid O Alahmedi, Sami Khairy, Salah Baz
Primary Angiitis of the central nervous system is a rare and poorly understood variant of vasculitis. We narrate a case of a 46-year-old male who presented with new onset refractory status epilepticus mimicking autoimmune encephalitis. In this case we are reporting clues that could be useful for diagnosis and extensive literature review on the topic.
2017: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/29061238/primary-angiitis-of-the-central-nervous-system
#2
REVIEW
Jennifer Mandal, Sharon A Chung
Primary angiitis of the central nervous system (PACNS) is rare but often included in the differential diagnosis for unusual or unexplained neurologic symptoms owing to its array of clinical manifestations. PACNS is not a single entity, but rather an umbrella term for distinct subtypes of disease affecting different parts of the CNS, different sized vessels, and with different histologic and pathologic findings. Although brain biopsy is considered the gold standard test, it is neither highly sensitive nor specific...
November 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29057849/a-closer-look-at-angiitis-of-the-central-nervous-system
#3
Cheng Wan, Hua Su
Angiitis of the central nervous system (ACNS) is a rather new disease entity that is defined as vascular inflammation limited to the central nervous system and was formally nominated in 1959. Etiologically, it can be divided into primary and secondary ACNS. However, the potential pathogenesis of ACNS remains unclear. The clinical presentation is variable, and there is no consensus concerning its diagnosis and management. Although the incidence is relatively low, ACNS is still a life-threatening condition. It is essential to get a comprehensive and updated understanding of the disease...
October 2017: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/29040748/primary-angiitis-of-the-central-nervous-system-a-territory-about-to-be-discovered
#4
Thomas Daikeler, Alan Tyndall
No abstract text is available yet for this article.
October 12, 2017: Rheumatology
https://www.readbyqxmd.com/read/28875326/neuroradiologic-characteristics-of-primary-angiitis-of-the-central-nervous-system-according-to-the-affected-vessel-size
#5
Christian Thaler, Ann-Katrin Kaufmann-Bühler, Tserenchunt Gansukh, Amarjargal Gansukh, Simon Schuster, Henrike Bachmann, Götz Thomalla, Tim Magnus, Jakob Matschke, Jens Fiehler, Susanne Siemonsen
INTRODUCTION: Magnetic resonance imaging (MRI) has an important impact in diagnosing primary angiitis of the central nervous system (PACNS). However, neuroradiologic findings may vary immensely, making an easy and definite diagnosis challenging. METHODS: In this retrospective, single center study, we analyzed neuroradiologic findings of patients with PACNS diagnosed at our hospital between 2009 and 2014. Furthermore, we classified patients according to the affected vessel size and compared imaging characteristics between the subgroups...
September 5, 2017: Clinical Neuroradiology
https://www.readbyqxmd.com/read/28864038/radiologically-isolated-cerebral-amyloid-angiopathy-related-inflammation
#6
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...
November 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28758039/primary-angiitis-of-the-center-nervous-system-a-clinical-challenge-diagnosed-postmortem
#7
Bayan Al Share, Ali Zakaria, Evan Hiner, Ziyad Iskenderian, Nader Warra
Primary angiitis of the central nervous system (PACNS) is a rare vasculitis involving medium and small blood vessels of the brain, spinal cord, and meninges, without systemic involvement. The diffuse and patchy nature of its pathology is reflected by a wide spectrum of nonspecific clinical symptoms. Diagnosis is challenging due to lack of defined clinical criteria or specific imaging findings. Specific workup should be done only after exclusion of other etiologies, including infectious, neoplastic, toxic, and other vascular etiologies including systemic vasculitis...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28705900/subtypes-of-primary-angiitis-of-the-cns-identified-by-mri-patterns-reflect-the-size-of-affected-vessels
#8
Simon Schuster, Henrike Bachmann, Vivien Thom, Ann-Katrin Kaufmann-Buehler, Jakob Matschke, Susanne Siemonsen, Markus Glatzel, Jens Fiehler, Christian Gerloff, Tim Magnus, Götz Thomalla
OBJECTIVE: To describe patterns of diagnostic findings, and identify subgroups of primary angiitis of the central nervous system (PACNS). METHODS: We retrospectively analysed 31 patients with PACNS. Cases were selected by predetermined diagnostic criteria and stratified into biopsy-proven and imaging-based PACNS. We compared clinical characteristics, cerebrospinal fluid (CSF) findings and imaging results including high-resolution vessel wall MRI between groups. RESULTS: There were 31 cases of PACNS (mean age 45...
September 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28672966/role-of-magnetic-resonance-imaging-in-the-diagnosis-of-primary-central-nervous-system-angiitis
#9
Lei Niu, Liwei Wang, Xindao Yin, Xiao-Feng Li, Feng Wang
The present study reported on the use of magnetic resonance imaging (MRI) in the diagnosis of primary angiitis of the central nervous system (PACNS). A total of 19 consecutive patients with a clinical diagnosis of PACNS confirmed by clinical follow-up were enrolled in the present study. All patients underwent unenhanced and enhanced MRI prior to and after steroids or steroids plus immunosuppressive therapy. At baseline, all patients showed lesions on MRI in the grey and white matter. Lesions presented as slightly hypointense on T1-weighted images (T1WI), slightly hyperintense on T2WI, hyperintense on fluid-attenuated inversion recovery, iso- or slightly hyperintense on diffusion-weighted images (DWI) and hyperintense on apparent diffusion coefficient (ADC) mapping...
July 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28570324/neurologic-complications-of-lymphoma-leukemia-and-paraproteinemias
#10
Michelle L Mauermann
PURPOSE OF REVIEW: This article reviews the spectrum of neurologic complications associated with lymphoma, leukemia, and paraproteinemic disorders. While leptomeningeal metastasis is the most common complication of lymphoma and leukemia and peripheral neuropathy is the most common complication of paraproteinemic disorders, clinicians need to be familiar with the diverse neurologic complications of these disorders. RECENT FINDINGS: Lymphomatous nervous system involvement can be difficult to diagnose, especially when it is the presenting symptom...
June 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28456915/reversible-cerebral-vasoconstriction-syndrome-recognition-and-treatment
#11
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
#12
Thomas Daikeler, Alan Tyndall
No abstract text is available yet for this article.
October 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28355722/-a-clinical-analysis-of-5-patients-with-infratentorial-primary-angiitis-of-central-nervous-system
#13
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
#14
MULTICENTER STUDY
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...
October 1, 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
#15
MULTICENTER STUDY
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
#16
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
#17
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
#18
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
#19
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)...
March 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/27864289/immunotherapy-for-arterial-ischaemic-stroke-in-childhood-a-systematic-review
#20
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
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