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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/29354215/the-role-of-biopsies-and-autopsies-in-the-diagnosis-of-cognitive-impairment-with-emphasis-on-small-vessel-diseases-a-critical-appraisal-enriched-by-personal-experience
#4
Leila Chimelli
Acquired and hereditary microangiopathies cause cerebral small vessel diseases (CSVD) that impair cognition. The most frequent is primary angiitis of the CNS (PACNS), whose diagnosis remains challenging, requiring a multidisciplinary approach. Secondary vasculitis, CADASIL, miscellaneous microangiopathies and lymphomas, also cause cognitive impairment. Despite the fact that the need for biopsy has decreased in the era of new neuroimaging methods, biopsies that include small leptomeningeal and parenchymal arterial vessels still remain the gold standard to diagnose PACNS and other CSVD, and to exclude mimics such as infections and malignancies...
October 2017: Dementia & Neuropsychologia
https://www.readbyqxmd.com/read/29328521/central-vein-sign-differentiates-multiple-sclerosis-from-central-nervous-system-inflammatory-vasculopathies
#5
Pietro Maggi, Martina Absinta, Matteo Grammatico, Luisa Vuolo, Giacomo Emmi, Giovanna Carlucci, Gregorio Spagni, Alessandro Barilaro, Anna Maria Repice, Lorenzo Emmi, Domenico Prisco, Vittorio Martinelli, Roberta Scotti, Niloufar Sadeghi, Gaetano Perrotta, Pascal Sati, Bernard Dachy, Daniel S Reich, Massimo Filippi, Luca Massacesi
OBJECTIVES: In multiple sclerosis (MS), magnetic resonance imaging (MRI) is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the "central vein sign") improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS-mimicking inflammatory and/or autoimmune diseases, such as central nervous system (CNS) inflammatory vasculopathies, is lacking...
February 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29061238/primary-angiitis-of-the-central-nervous-system
#6
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/28875326/neuroradiologic-characteristics-of-primary-angiitis-of-the-central-nervous-system-according-to-the-affected-vessel-size
#7
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/28765346/pitfalls-of-pacns-a-rare-case-of-central-nervous-system-vasculitis-associated-with-toxoplasmosis
#8
Hua-Xia Yang, Hong-Rui Liu, Feng-Chun Zhang
No abstract text is available yet for this article.
August 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28758039/primary-angiitis-of-the-center-nervous-system-a-clinical-challenge-diagnosed-postmortem
#9
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
#10
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
#11
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/28456915/reversible-cerebral-vasoconstriction-syndrome-recognition-and-treatment
#12
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/28413538/primary-cns-vasculitis-masquerading-as-glioblastoma-a-case-report-and-review
#13
Pelluru Pavan Kumar, Alugolu Rajesh, Rukmini Mrudula Kandadai, Aniruddh Kumar Purohit, Challa Sundaram
Isolated angitis of the central nervous system (IACNS)/primary angitis of central nervous system vasculitis (PACNS) is an uncommon vascular disease, sparingly presenting as an isolated inflammatory lesion on magnetic resonance imaging (MRI). The disease usually manifests as a long-drawn and progressive ischemic event. Delay in diagnosis due to focal nature of the lesion also contributes to the poor prognosis as the dismal natural history and immunosuppressive therapy. To date, only a few cases with tumor-like isolated angitis of CNS have been reported with clear and definitive diagnostic workup...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28355722/-a-clinical-analysis-of-5-patients-with-infratentorial-primary-angiitis-of-central-nervous-system
#14
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
#15
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/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/28243503/hemorrhagic-primary-cns-angiitis-and-vasoconstrictive-drug-exposure
#17
Mehmet A Topcuoglu, Ruchira M Jha, Jacob George, Matthew P Frosch, Aneesh B Singhal
BACKGROUND: Primary angiitis of the CNS (PACNS) typically manifests with accumulating neurologic deficits from ischemic strokes. Intracerebral hemorrhage (ICH) is an uncommon complication. There is limited knowledge about the risk factors and features of hemorrhagic PACNS. METHODS: We identified 49 patients (20 biopsy-proven) with PACNS diagnosed at our hospital from 1993 to 2015. We compared the features of hemorrhagic and nonhemorrhagic PACNS and analyzed the hemorrhagic PACNS cases in detail...
February 2017: Neurology. Clinical Practice
https://www.readbyqxmd.com/read/27940585/adult-primary-angiitis-of-the-central-nervous-system-isolated-small-vessel-vasculitis-represents-distinct-disease-pattern
#18
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/27570401/primary-cns-vasculitis-presenting-as-intraventricular-bleeding
#19
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/27432677/other-noninfectious-inflammatory-disorders
#20
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
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