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primary angiitis of the central nervous system

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https://www.readbyqxmd.com/read/29740727/the-first-case-of-bacillus-calmette-gu%C3%A3-rin-induced-small-vessel-central-nervous-system-vasculitis
#1
REVIEW
Marc-Etienne Parent, Maxime Richer, Patrick Liang
To present an unrecognized vascular complication of bacillus Calmette-Guérin (BCG) therapy administered for superficial bladder carcinoma. We also review the potential mimickers for primary angiitis of the central nervous system (PACNS) as well as complications of intravesical BCG therapy. An 89-year-old Caucasian man with a history of relapsing high-grade bladder carcinoma treated with intravesical BCG presented with recurring episodes of right upper limb paresthesia with clumsiness and dysarthria. Magnetic resonance imaging of the head revealed multiple predominantly left-sided frontotemporal micronodular peri-vascular lesions...
May 9, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29722896/cerebrospinal-fluid-findings-in-reversible-cerebral-vasoconstriction-syndrome-a-way-to-differentiate-from-cerebral-vasculitis
#2
Laura Kraayvanger, Peter Berlit, Philipp Albrecht, Hans-Peter Hartung, Markus Kraemer
INTRODUCTION: Reversible Cerebral Vasoconstriction Syndrome (RCVS) is characterized by an acute onset of severe headache and multifocal segmental vasoconstriction of cerebral arteries resolving within twelve weeks. Diagnostic criteria include normal or near-normal findings in cerebrospinal fluid (CSF) analysis, especially leukocyte levels < 10/mm³. Distinguishing RCVS from primary angiitis of the central nervous system (PACNS) is essential to avoid unnecessary and sometimes unfavorable immunosuppressive treatment...
May 3, 2018: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/29656658/primary-angiitis-of-the-central-nervous-system-mimicking-a-cerebellar-tumor
#3
Guodong Zhang, Chunxia Yang, Jinjin Chang, Xunhui Yuan, Yun'an Bai, Jianyi Niu, Meiying Wang
Primary angiitis of the central nervous system (PACNS), is a rare and poorly understood disease mainly characterized by multifocal segmental inflammation of the small and medium vessels of the central nervous system. Most PACNS are multiple lesions, occurring in the supratentorial subcortical and deep white matter, and only a few cases present as a tumor-like mass lesion. Herein, we describe an extremely rare case of PACNS occurred in the cerebellum, which mimicked a cerebellar tumor. To the best of our knowledge, this is the first reported case of cerebellar tumor-like PACNS proved by histopathological examination...
April 14, 2018: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29581340/arterial-wall-imaging-in-pediatric-stroke
#4
Nomazulu Dlamini, Ivanna Yau, Prakash Muthusami, David J Mikulis, Jorina Elbers, Mahmoud Slim, Rand Askalan, Daune MacGregor, Gabrielle deVeber, Manohar Shroff, Mahendranath Moharir
BACKGROUND AND PURPOSE: Arteriopathy is common in childhood arterial ischemic stroke (AIS) and predicts stroke recurrence. Currently available vascular imaging techniques mainly image the arterial lumen rather than the vessel wall and have a limited ability to differentiate among common arteriopathies. We aimed to investigate the value of a magnetic resonance imaging-based technique, namely noninvasive arterial wall imaging (AWI), for distinguishing among arteriopathy subtypes in a consecutive cohort of children presenting with AIS...
April 2018: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29429204/-small-vessel-childhood-primary-angiitis-of-the-central-nervous-system-a-case-report-and-literature-review
#5
REVIEW
J Deng, F Fang, X H Wang, M Ge, L J He, N Zhang
Objective: To summarize the clinical and pathological features of small vessel-childhood primary angiitis of the central nervous system (SV-cPACNS), discuss the immune therapy and increase the attention to brain biopsy in SV-cPACNS. Methods: The clinical data, pathology of brain biopsy, treatment and outcome of an SV-cPACNS patient hospitalized in Beijing Children's Hospital in February 2016 were analyzed retrospectively. The cases reported at Pubmed, CNKI and Wanfang databases from 2007 to 2017 were searched, the clinical and pathological features, immunotherapy and prognosis of the disease were summarized according to the literature review...
February 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29369829/primary-angiitis-of-the-central-nervous-system-mimicking-sporadic-creutzfeldt-jakob-disease-a-case-study
#6
Dian He, Gang Cai, Yan Li, Qi Liu, Kang Xiao, ChunTing Liu, Lan Chu, Shan Wu, Fang Liu, MingYao You, WeiJia Jiang
No abstract text is available yet for this article.
January 29, 2018: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/29062691/new-onset-refractory-status-epilepticus-due-to-primary-angiitis-of-the-central-nervous-system
#7
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
#8
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
#9
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
#10
Thomas Daikeler, Alan Tyndall
No abstract text is available yet for this article.
December 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28875326/neuroradiologic-characteristics-of-primary-angiitis-of-the-central-nervous-system-according-to-the-affected-vessel-size
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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