332 results match your criteria Primary Angiitis of the CNS


Neurological and neuropsychiatric complications of COVID-19 in 153 patients: a UK-wide surveillance study.

Lancet Psychiatry 2020 Jun 25. Epub 2020 Jun 25.

The National Institute for Health Research Health Protection Research Unit for Emerging and Zoonotic Infections, Liverpool, UK; Department of Clinical Infection Microbiology and Immunology, Institute of Infection, Veterinary, and Ecological Sciences, University of Liverpool, Liverpool, UK; The Walton Centre NHS Foundation Trust, Liverpool, UK. Electronic address:

Background: Concerns regarding potential neurological complications of COVID-19 are being increasingly reported, primarily in small series. Larger studies have been limited by both geography and specialty. Comprehensive characterisation of clinical syndromes is crucial to allow rational selection and evaluation of potential therapies. Read More

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http://dx.doi.org/10.1016/S2215-0366(20)30287-XDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7316461PMC

The rare case of a 20-year-old male with rapidly progressive primary angiitis of the CNS with a good outcome.

Neurol Neuroimmunol Neuroinflamm 2020 Sep 9;7(5). Epub 2020 Jun 9.

From the Department of Neurological Sciences (E.G., T.S., D.S., R.K.G.), Department of Radiology (M.K.) and Department of Pathology (A.N.), Rush University Medical Center, Chicago, IL.

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http://dx.doi.org/10.1212/NXI.0000000000000793DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7309525PMC
September 2020

Primary CNS lymphoma initially diagnosed as vasculitis.

Neurol Clin Pract 2020 Feb;10(1):84-88

Neurological Institute, Cleveland Clinic, OH.

Purpose Of Review: A vasculitic pattern of injury seen on brain biopsy can be attributed to a multitude of primary or secondary disorders, leading to diagnostic challenges for clinicians.

Recent Findings: This report describes the clinical presentation and histopathologic findings in 2 patients who initially received a diagnosis of primary CNS vasculitis, but did not show long-term response to treatment. In both cases, a second biopsy was performed, and the final diagnosis was primary CNS lymphoma (PCNSL). Read More

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http://dx.doi.org/10.1212/CPJ.0000000000000693DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7057073PMC
February 2020

An ischemic stroke as the presenting manifestation of rapidly progressive primary angiitis of central nervous system in a 17-year-old boy.

J Neuroimmunol 2020 Apr 13;341:577190. Epub 2020 Feb 13.

Department of Neurology, Patras University Hospital, Patras, Greece.

Background: Childhood primary angiitis of the central nervous system (cPACNS) is an increasingly recognized inflammatory brain disease in children.

Case Presentation: We present a case of a 17-year-old boy with recurrent ischemic events over a short time period. Diagnosis of angiography positive cPACNS was made based on neuroimaging findings while secondary causes or mimics of CNS vasculitis were meticulously excluded. Read More

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http://dx.doi.org/10.1016/j.jneuroim.2020.577190DOI Listing

Pearls & Oy-sters: Primary angiitis of the CNS presenting with recurrent intracranial hemorrhage.

Neurology 2020 03 10;94(9):e992-e995. Epub 2020 Feb 10.

From the Department of Neurology, Oregon Health & Science University, Portland.

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http://dx.doi.org/10.1212/WNL.0000000000009047DOI Listing

Cerebellar PACNS in an Elderly Patient Present as a Tumor-Like Mass Lesion: A case Report.

Acta Neurol Taiwan 2019 Jun;28(2):38-43

Division of Neurology, Department of Medicine, Kaohsiung Veterans General Hospital, Taiwan.

Purpose: PACNS has a broad spectrum of clinical manifestations without typical features, and its clinical diagnosis is challenging. We report an elderly patient of cerebellar PACNS (Primary angiitis of central nervous system) presented as a brain tumor by MRI, and primary angiitis was proven by pathology.

Case Report: We report an 81-year-old female who complained about vertigo for 3 weeks with right arm dysmetria. Read More

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Spatial Distribution of Intracranial Vessel Wall Enhancement in Hypertension and Primary Angiitis of the CNS.

Sci Rep 2019 12 17;9(1):19270. Epub 2019 Dec 17.

Division of Neuroradiology, Massachusetts General Hospital, Harvard Medical School, Massachusetts, USA.

We hypothesized a difference in the spatial distribution of intracranial vessel wall enhancement between CNS vasculitis and risk factors for intracranial atherosclerotic disease (ICAD). Fifty-five vessel wall MR imaging (VWI) exams were included in this retrospective observational study. Intracranial arteries were evaluated for vessel wall enhancement by branching pattern (e. Read More

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http://dx.doi.org/10.1038/s41598-019-55634-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6917817PMC
December 2019

Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review.

Front Neurol 2019 13;10:1208. Epub 2019 Nov 13.

Department of Neurology, Stroke Center, The First Hospital of Jilin University, Changchun, China.

Primary angiitis of the central nervous system (PACNS) is a rare disorder resulting in idiopathic inflammation affecting the parenchymal and leptomeningeal vessels confined to the central nervous system (CNS), of which a tumor-like mass lesion is an even rare subtype. We described a case of PACNS initially misdiagnosed as glioblastoma. The patient was a 35 year-old female with right-sided weakness and expressive dysphasia. Read More

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http://dx.doi.org/10.3389/fneur.2019.01208DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6863980PMC
November 2019

Neuroimaging of Multiple Sclerosis Mimics.

Neurol Clin 2020 02 24;38(1):149-170. Epub 2019 Oct 24.

Dent Neurological Institute, Buffalo, NY, USA.

Multiple sclerosis (MS) is the most common immune-mediated disease of the central nervous system, characterized by demyelinating lesions of the brain and the spinal cord. Although it is extremely important to diagnose this condition in a timely manner, to initiate and monitor treatment to prevent permanent neurologic damage and disability, it is also necessary that other demyelinating conditions collectively referred to as MS mimics be identified and excluded. This article describes the in-depth neuroimaging characteristics and morphology of the pathologic lesions on the various neuroimaging modalities. Read More

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http://dx.doi.org/10.1016/j.ncl.2019.09.002DOI Listing
February 2020
5 Reads

The diagnosis of primary central nervous system vasculitis.

Pract Neurol 2020 Apr 24;20(2):109-114. Epub 2019 Oct 24.

Institute of Clinical Neurosciences, University of Bristol, Bristol, UK

The diagnosis of primary central nervous system (CNS) vasculitis is often difficult. There are neither specific clinical features nor a classical clinical course, and no blood or imaging investigations that can confirm the diagnosis. Contrast catheter cerebral angiography is neither specific nor sensitive, yet still underpins the diagnosis in many published studies. Read More

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http://dx.doi.org/10.1136/practneurol-2018-002002DOI Listing

Varicella-zoster virus cns disease clinical features in ukrainian patients. prospective study.

Wiad Lek 2019 ;72(9 cz 2):1765-1768

Center of Infectious Disorders of the Nervous System, Si "L.V. Gromashevsky Institute of Epidemiology and Infection Diseases of Nams of Ukraine", KYIV, UKRAINE.

Objective: Introduction: Herpes zoster (HZ), or shingles, is localized disease characterized by unilateral radicular pain and a vesicular rash limited to the area of skin innervated by a single dorsal root or cranial sensory ganglion. Whereas varicella, or chickenpox, results from primary exogenous varicella-zoster virus (VZV) infection, HZ is caused by reactivation of endogenous VZV that has persisted in latent form within sensory ganglia following an earlier episode of chickenpox. The aim: To explore the clinical features, diagnosis, and treatment of CNS injury caused by VZV infection in a prospective single center study from January 2014 to January 2018. Read More

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November 2019
1 Read

Central Nervous System Vasculopathies.

Radiol Clin North Am 2019 Nov 16;57(6):1117-1131. Epub 2019 Aug 16.

Department of Neuroradiology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114, USA.

Various imaging techniques play a role in the diagnosis of CNS vasculopathies, which comprise a heterogeneous group of disorders, including various noninflammatory and inflammatory etiologies. Noninflammatory vasculopathies include entities such as CADASIL, Susac, moyamoya, fibromuscular dysplasia, vasculopathy of connective tissue disorders, and reversible vasoconstriction syndrome. Inflammatory vasculopathies include vasculitides of different vessel sizes, primary angiitis of the CNS, vasculitis of systemic disease, and vasculitis secondary to specific causes. Read More

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http://dx.doi.org/10.1016/j.rcl.2019.07.005DOI Listing
November 2019
1 Read

Seizures, Confusion, and Strokes in a Patient With AIDS.

Neurohospitalist 2019 Oct 14;9(4):209-214. Epub 2019 Feb 14.

Department of Neurology and Neurosciences, Stanford School of Medicine, Stanford, CA, USA.

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http://dx.doi.org/10.1177/1941874419830499DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6739673PMC
October 2019

Concordance of angiography and cerebral biopsy results for suspected primary central nervous system vasculitis: A multi-center retrospective review.

Clin Neurol Neurosurg 2019 Oct 9;185:105482. Epub 2019 Aug 9.

Case Western Reserve University School of Medicine, Cleveland, OH, United States; Department of Neurology, University Hospitals Cleveland Medical Center, Cleveland OH, United States. Electronic address:

Objective: Primary CNS Vasculitis (PCNSV) is a rare disease that is often challenging to diagnose. Cerebral angiography and biopsy have been utilized in the diagnostic workup for several decades but limited literature reports on the concordance of findings of angiography and biopsy. The primary objective of this work was to examine how cerebral angiography corresponded with biopsy findings in patients with suspected PCNSV. Read More

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http://dx.doi.org/10.1016/j.clineuro.2019.105482DOI Listing
October 2019
2 Reads

Strategies for treatment of childhood primary angiitis of the central nervous system.

Neurol Neuroimmunol Neuroinflamm 2019 07 3;6(4):e567. Epub 2019 May 3.

Cumming School of Medicine (J.B., S.M.B., A.D.), University of Calgary, Alberta, Canada; Section of Rheumatology (S.M.B., M.T.), Department of Pediatrics, Alberta Children's Hospital, Alberta Children's Hospital Research Institute, Calgary, Alberta, Canada; and University of Calgary (B.G.), Alberta, Canada.

Objective: Childhood primary angiitis of the CNS (cPACNS) is a devastating neurologic disease. No standardized treatment protocols exist, and evidence is limited to open-label cohort studies and case reports. The aim of this review is to summarize the literature and provide informed treatment recommendations. Read More

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http://nn.neurology.org/lookup/doi/10.1212/NXI.0000000000000
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http://dx.doi.org/10.1212/NXI.0000000000000567DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6624095PMC
July 2019
4 Reads

Leptomeningeal metastases with subarachnoid hemorrhage mimicking primary angiitis of CNS.

Neurol India 2019 May-Jun;67(3):856-858

Department of Neurosurgery, Aster Medcity, Kothad, Kochi, Kerala, India.

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http://dx.doi.org/10.4103/0028-3886.263252DOI Listing
March 2020
2 Reads

Exploratory proteomic analysis implicates the alternative complement cascade in primary CNS vasculitis.

Neurology 2019 07 3;93(5):e433-e444. Epub 2019 Jul 3.

From the Departments of Biochemistry and Biophysics (C.M.-B., H.R., H.A.S., K.C.Z., J.L.D.), Pharmaceutical Chemistry (G.M.K., A.Y.), Pathology and Laboratory Medicine (T.T.), and Neurology (S.A.J., V.C.D., J.M.G., M.R.W.), University of California, San Francisco; Department of Rheumatology/Immunology (R.A.H.-A., L.H.C.), Cleveland Clinic, OH; Department of Neurology (M.P.G.), Boston Children's Hospital, MA; Division of Neurology (J.M.C.), Connecticut Children's Medical Center, Hartford; Division of Rheumatology (A.G.S.), University of Pennsylvania, Philadelphia; Kaiser Permanente (J.F.M.), San Francisco Medical Center; UCSF Weill Institute for Neurosciences (S.A.J., V.C.D., J.M.G., M.R.W.); and Chan Zuckerberg Biohub (J.L.D.), San Francisco, CA.

Objective: To identify molecular correlates of primary angiitis of the CNS (PACNS) through proteomic analysis of CSF from a biopsy-proven patient cohort.

Methods: Using mass spectrometry, we quantitatively compared the CSF proteome of patients with biopsy-proven PACNS (n = 8) to CSF from individuals with noninflammatory conditions (n = 11). Significantly enriched molecular pathways were identified with a gene ontology workflow, and high confidence hits within enriched pathways (fold change >1. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007850DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6693432PMC
July 2019
4 Reads
8.286 Impact Factor

Primary Angiitis of the Central Nervous System: New Potential Imaging Techniques and Biomarkers in Blood and Cerebrospinal Fluid.

Front Neurol 2019 6;10:568. Epub 2019 Jun 6.

Department of Neurology, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany.

Primary angiitis of the central nervous system (PACNS) is an inflammatory brain disease affecting the medium and small vessels of the CNS. Although recent data of patients with PACNS have advanced the understanding of the disease, the diagnosis remains challenging. Clinical presentation of PACNS is broad and unspecific and the majority of the diagnostic approaches are hallmarked by a low specificity. Read More

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http://dx.doi.org/10.3389/fneur.2019.00568DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6562270PMC
June 2019
5 Reads

Cerebral amyloid-β-related angiitis without cerebral microbleeds in a patient with subarachnoid hemorrhage.

Cardiovasc Pathol 2019 Sep - Oct;42:36-40. Epub 2019 May 25.

Department of Diagnostic Pathology, NTT Medical Center Tokyo, Tokyo, Japan; Faculty of Healthcare, Tokyo Healthcare University, Tokyo, Japan.

Amyloid-β-related angiitis (ABRA), a subtype of cerebral amyloid angiopathy (CAA), is vasculitis occurring in relation to amyloid-β (Aβ) deposition in the walls of intracranial blood vessels. ABRA is presumed to be caused by some immune response to the deposited Aβ. An 81-year-old man on oral anticoagulant therapy complained of headache, nausea, and difficulty with standing after a head injury. Read More

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http://dx.doi.org/10.1016/j.carpath.2019.05.004DOI Listing
December 2019
6 Reads

Multiple subacute bilateral cerebral infarcts in a 9-year-old Nigerian male: a case of childhood primary angiitis of the central nervous system.

Pan Afr Med J 2018 10;31:99. Epub 2018 Oct 10.

Children's Emergency Room (CHER), Ward 12 (children's in-patient ward) and the Paediatric Neurology Clinic, Federal Medical Centre, Owerri, Nigeria.

A Primary Angiitis of the Central Nervous System (PACNS) is ill-defined, complex and rare in children. Clinical presentation is variable, diagnosis is challenging and it is life-threatening but treatable. The index case is a 9-year old male who presented with progressively slurred speech, progressive weakness of the limbs, hemifacial weakness and vomiting. Read More

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http://dx.doi.org/10.11604/pamj.2018.31.99.13898DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6462305PMC
April 2019
8 Reads

X-Linked Lymphoproliferative Syndrome Presenting as Adult-Onset Multi-Infarct Dementia.

J Neuropathol Exp Neurol 2019 05;78(5):460-466

Department of Neurology, Mayo Clinic, Jacksonville, Florida.

Pathogenic hemizygous variants in the SH2D1A gene cause X-linked lymphoproliferative (XLP) syndrome, a rare primary immunodeficiency usually associated with fatal Epstein-Barr virus infection. Disease onset is typically in early childhood, and the average life expectancy of affected males is ∼11 years. We describe clinical, radiographic, neuropathologic, and genetic features of a 49-year-old man presenting with central nervous system vasculitis that was reminiscent of adult primary angiitis but which was unresponsive to treatment. Read More

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https://academic.oup.com/jnen/article/78/5/460/5445919
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http://dx.doi.org/10.1093/jnen/nlz018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467195PMC
May 2019
44 Reads
3.797 Impact Factor

Biopsy-proven primary angiitis of the central nervous system mimicking leukodystrophy: A case report and review of the literature.

J Clin Neurosci 2019 Jun 22;64:42-44. Epub 2019 Mar 22.

Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico "C. Besta", Via Celoria 11, 20133 Milan, Italy.

Primary Angiitis of the Central Nervous System (PACNS) is a rare form of idiopathic CNS vasculitis. Neuroimaging is often abnormal and characterized by multifocal brain lesions, but brain biopsy definitely confirms the diagnosis. We report the rare case of a 45-year-old female presenting with symptoms of intracranial hypertension and leukodystrophy-like neuroimaging findings. Read More

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http://dx.doi.org/10.1016/j.jocn.2019.03.021DOI Listing
June 2019
10 Reads

Relapse rates and long-term outcome in primary angiitis of the central nervous system.

J Neurol 2019 Jun 23;266(6):1481-1489. Epub 2019 Mar 23.

Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Objective: To analyze the treatment response in patients with primary angiitis of the central nervous system (PACNS).

Methods: In a single-center retrospective observational study, we assessed relapses, remission, and long-term outcome by use of the modified Rankin Scale (mRS) under different immunotherapies. Eligible patients had CNS biopsy in favor of PACNS or neuroimaging compatible with PACNS after exclusion of an alternative diagnosis. Read More

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http://dx.doi.org/10.1007/s00415-019-09285-1DOI Listing
June 2019
8 Reads

Primary angiitis of the central nervous system: Clinical profiles and outcomes of 45 patients.

Neurol India 2019 Jan-Feb;67(1):105-112

Comprehensive Stroke Care Program, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.

Objective: To describe the clinical profile, treatment response and predictors of outcome in patients with primary angiitis of the central nervous system (PACNS) from a single tertiary care center.

Methodology: Retrospective analysis of consecutive patients diagnosed with PACNS from January 2000 to December 2015. Outcome was defined as poor when the 6-month modified Rankin scale (mRS) was ≥3. Read More

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http://dx.doi.org/10.4103/0028-3886.253578DOI Listing
December 2019
36 Reads
1.084 Impact Factor

Anti-MOG encephalitis mimicking small vessel CNS vasculitis.

Neurol Neuroimmunol Neuroinflamm 2019 03 1;6(2):e538. Epub 2019 Feb 1.

Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.

Objective: To report 2 patients with anti-myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis who were initially misdiagnosed with small vessel primary CNS vasculitis.

Methods: Review of symptoms, MRI and neuropathologic features, and response to treatment. MOG antibodies were determined in serum and CSF using a cell-based assay. Read More

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http://dx.doi.org/10.1212/NXI.0000000000000538DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6384022PMC
March 2019
11 Reads

Cognitive outcomes of childhood primary CNS vasculitis.

Neuropsychology 2019 May 14;33(4):462-469. Epub 2019 Feb 14.

Alberta Children's Hospital Research Institute, Department of Pediatrics, Cumming School of Medicine, University of Calgary.

Objective: To characterize the clinical cognitive phenotypes and severity of cognitive burden according to disease subtype in children with primary central nervous system vasculitis (cPACNS).

Method: This retrospective multicenter inflammatory brain disease database study examined the neuropsychological outcomes of 80 children (44 male; mean age = 7.89 years, SD = 4. Read More

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http://doi.apa.org/getdoi.cfm?doi=10.1037/neu0000513
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http://dx.doi.org/10.1037/neu0000513DOI Listing
May 2019
39 Reads

MOG antibody disease manifesting as progressive cognitive deterioration and behavioral changes with primary central nervous system vasculitis.

Mult Scler Relat Disord 2019 May 2;30:48-50. Epub 2019 Feb 2.

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. Electronic address:

We report a 60-year-old male with anti-myelin oligodendrocyte glycoprotein (MOG) antibody who developed progressive cognitive deterioration and behavioral changes, with no other focal signs, over 9 months. MRI showed numerous T2-hyperintense lesions with partial contrast enhancement in white and grey matter of cerebrum, cerebellum and spinal cord. A brain biopsy revealed perivascular inflammatory cell infiltration, disturbed vascular continuity and no demyelination, indicative of a lymphocytic pattern of primary CNS vasculitis (PCNSV). Read More

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http://dx.doi.org/10.1016/j.msard.2019.01.053DOI Listing
May 2019
3 Reads

Atypical presentation of fulminant primary central nervous system angiitis.

J Neuroimmunol 2019 05 1;330:1-4. Epub 2019 Feb 1.

Department of Critical Care Medicine, University of Calgary, Calgary, AB T2N 1N4, Canada. Electronic address:

Background: Primary Angiitis of the Central Nervous System (PACNS) is a rare cause of CNS vasculitis that should be included as part complete differential diagnosis, especially in cases with suggestive imaging findings and an absence of secondary causes for CNS vasculitis.

Case Presentation: We describe a case of a 47-year-old previously healthy Caucasian male presenting with rapid progression of encephalopathy and fevers. Extensive infectious, autoimmune, and imaging workups were unrevealing. Read More

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http://dx.doi.org/10.1016/j.jneuroim.2019.01.019DOI Listing
May 2019
5 Reads

RCVS score and diagnostic approach for reversible cerebral vasoconstriction syndrome.

Neurology 2019 02 11;92(7):e639-e647. Epub 2019 Jan 11.

From the Department of Neurology (E.A.R., A.B.S.), Massachusetts General Hospital, Boston; Department of Neurology (E.A.R., G.S.S.), Universidade Federal de São Paulo, Brazil; Hospital Israelita Albert Einstein (G.S.S.), São Paulo, Brazil; and Department of Neurology (M.A.T.), Hacettepe University, Ankara, Turkey.

Objective: To develop a method to distinguish reversible cerebral vasoconstriction syndrome (RCVS) from other large/medium-vessel intracranial arteriopathies.

Methods: We identified consecutive patients from our institutional databases admitted in 2013-2017 with newly diagnosed RCVS (n = 30) or non-RCVS arteriopathy (n = 80). Admission clinical and imaging features were compared. Read More

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http://dx.doi.org/10.1212/WNL.0000000000006917DOI Listing
February 2019
24 Reads

Anti-neutrophil cytoplasmic antibody associated central nervous system vasculitis with brain and spinal cord subarachnoid hemorrhage: A rare case report and review of the literature.

J Clin Neurosci 2019 Apr 26;62:253-255. Epub 2018 Dec 26.

Department of Neurosurgery, United States. Electronic address:

This case report describes a 48-year old female who presented with altered mental status, lower extremity weakness, low back pain and a recent history of subjective fevers and night sweats found to have posterior parieto-occipital and spinal subarachnoid hemorrhage on imaging. Further work-up revealed vasculitic changes in the intracranial vasculature and the external carotid artery on angiography. She also demonstrated positivity for perinuclear anti-neutrophil cytoplasmic (p-ANCA) antibodies overall consistent with ANCA associated central nervous system vasculitis (AAV). Read More

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http://dx.doi.org/10.1016/j.jocn.2018.12.001DOI Listing
April 2019
10 Reads

Health-related quality of life in children with inflammatory brain disease.

Pediatr Rheumatol Online J 2018 Nov 20;16(1):73. Epub 2018 Nov 20.

Rheumatology, Department of Pediatrics, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, T3B 6A8, Canada.

Objective: To quantify the impact of inflammatory brain diseases in the pediatric population on health-related quality of life, including the subdomains of physical, emotional, school and social functioning.

Methods: This was a multicenter, observational cohort study of children (< 18 years of age) diagnosed with inflammatory brain disease (IBrainD). Patients were included if they had completed at least one Health Related Quality of Life Questionnaire (HRQoL). Read More

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http://dx.doi.org/10.1186/s12969-018-0291-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245877PMC
November 2018
49 Reads

High-resolution Intracranial Vessel Wall Imaging in Monitoring Treatment Response in Primary CNS Angiitis.

Neurologist 2018 Nov;23(6):188-190

Second Department of Neurology, School of Medicine, "Attikon" University Hospital, National and Kapodistrian University of Athens.

Introduction: High-resolution vessel wall imaging (HR-VWI) is emerging as a tool of notable utility in the diagnosis of intracranial vessel pathology. Its role in monitoring vessel wall disease response to treatment, however, is less well-established.

Case Report: We report the case of a 45-year-old man with left middle and anterior cerebral artery infarcts and an National Institute of Health Stroke Scale (NIHSS) score of 2. Read More

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http://dx.doi.org/10.1097/NRL.0000000000000198DOI Listing
November 2018
14 Reads

Intracranial Atherosclerosis Versus Primary Angiitis of the Central Nervous System: a Case Report.

Cureus 2018 Jul 23;10(7):e3031. Epub 2018 Jul 23.

Ross University School of Medicine, California Hospital Medical Center, Los Angeles, USA.

Primary angiitis of the central nervous system (PACNS) is a rare disease with various clinical presentations. It is the preferred name for vasculitis that is confined to the central nervous system (CNS) and is often considered a diagnosis of exclusion in vascular or inflammatory CNS diseases. This case describes a 46-year-old right-handed female with a past medical history of hypertension (HTN), hyperlipidemia (HLD), diabetes mellitus type two (DM2), obesity, and hemorrhagic stroke who was transferred from an outside facility after a one-month hospitalization to be evaluated for CNS vasculitis. Read More

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http://dx.doi.org/10.7759/cureus.3031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6150750PMC
July 2018
10 Reads

Primary angiitis of the CNS and reversible cerebral vasoconstriction syndrome: A comparative study.

Neurology 2018 10 19;91(16):e1468-e1478. Epub 2018 Sep 19.

From the Department of Internal Medicine (H.d.B., A.A.), Caen University Hospital; University of Caen-Normandie (H.d.B., E.T., A.A); Biostatistics and Clinical Research Unit (J.-J.P.), Caen University Hospital; Emergency Headache Centre (J.M., C.B.), Lariboisière Hospital, Assistance Publique des Hôpitaux de Paris; Department of Neurology (C.A., A.D.), Montpellier University Hospital; Department of Neuroradiology (G.B., O.N.), Sainte-Anne Hospital, Paris-Descartes University; INSERM UMR 894 (O.N.); Department of Vascular Neurology (M.Z.), Saint Joseph Hospital; Université Paris-Descartes (M.Z.), INSERM UMR S 919, Paris; Department of Neurology (E.T.), Caen University Hospital; University of Caen-Normandie (E.T.), INSERM U919; Department of Neurology (M.-G.B.), Lariboisière Hospital, Assistance Publique des Hôpitaux de Paris, France; and Division of Rheumatology (C.P.), Vasculitis Clinic, Mount Sinai Hospital, Toronto, Ontario, Canada.

Objectives: To further improve the distinction between primary angiitis of the CNS (PACNS) and reversible cerebral vasoconstriction syndrome (RCVS).

Methods: We compared 2 large French cohorts of patients with PACNS (n = 110, retrospectively and prospectively enrolled) and RCVS (n = 173, prospectively enrolled).

Results: Patients with RCVS were predominantly female ( < 0. Read More

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http://dx.doi.org/10.1212/WNL.0000000000006367DOI Listing
October 2018
53 Reads
8.290 Impact Factor

Diagnosis and Treatment of Primary Central Nervous System Angiitis.

Curr Treat Options Neurol 2018 Aug 4;20(9):38. Epub 2018 Aug 4.

Neurology, University of Iowa, Iowa City, IA, USA.

Purpose Of Review: Primary central nervous system angiitis (PCNSA) is a rare disease. Varying clinical pictures coupled with lack of sensitive and specific diagnostic tests lead to challenges in management of these patients. This unfortunately may lead to both under- or over-diagnosis and unnecessary treatment. Read More

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http://dx.doi.org/10.1007/s11940-018-0522-5DOI Listing
August 2018
23 Reads

Amyloid-Beta-Related Angiitis with Distinctive Neuro-Ophthalmologic Features.

Neuroophthalmology 2018 Aug 19;42(4):237-241. Epub 2017 Sep 19.

Department of Neurology, Mayo Clinic, Phoenix, Arizona, USA.

Amyloid beta-related angiitis (ABRA) is a subtype of cerebral amyloid angiopathy-related inflammation, with distinctive pathology and prognosis compared with cerebral amyloid angiopathy (CAA). On a spectrum of increasing severity, ABRA is considered to be in-between the less aggressive inflammatory-CAA and the more severe primary central nervous system (CNS) angiitis. Whereas retinal pathological changes were described in subjects with primary or secondary CNS angiitis, and non-inflammatory CAA, bilateral posterior pole superficial and peripapillary retinal hemorrhages have not been reported as initial signs in patients with pathology-confirmed ABRA, accompanying neurological spells and characteristic neuroimaging findings. Read More

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http://dx.doi.org/10.1080/01658107.2017.1374982DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6056207PMC
August 2018
13 Reads

Primary optic neuropathy in Behçet's syndrome.

Mult Scler 2019 07 9;25(8):1132-1140. Epub 2018 Jul 9.

Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.

Background: Primary optic neuropathy in Behçet's syndrome (PONBS) is limited to a few case reports.

Objective: To investigate the clinical features, magnetic resonance imaging (MRI) changes, and visual prognosis of PONBS.

Methods: Sixty-one patients who presented with first onset of optic neuritis and fulfilled the International Criteria for Behçet's Disease (ICBD) were evaluated. Read More

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http://dx.doi.org/10.1177/1352458518786058DOI Listing
July 2019
12 Reads
4.820 Impact Factor

[Misleading cerebral arterial wall gadolinium-enhancement in malignant lymphoma].

Rinsho Shinkeigaku 2018 Jul 30;58(7):456-459. Epub 2018 Jun 30.

Department of Neurology, Tosei General Hospital.

A previously healthy, 77-year-old woman presented with gradual cognitive decline and acute gait imbalance. On admission, despite no obvious paralysis, she tilted to the right. Her Mini-Mental State Examination score was slightly low (23/30). Read More

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http://dx.doi.org/10.5692/clinicalneurol.cn-001173DOI Listing
July 2018
43 Reads

Immune cell profiling in the cerebrospinal fluid of patients with primary angiitis of the central nervous system reflects the heterogeneity of the disease.

J Neuroimmunol 2018 08 7;321:109-116. Epub 2018 Jun 7.

Clinic of Neurology with Institute of Translational Neurology, University Hospital Münster, University Münster, 48149 Münster, Germany. Electronic address:

Primary angiitis of the central nervous system (PACNS) is a rare and heterogeneous inflammatory disease of the CNS vasculature with poorly understood pathophysiology. Comprehensive immune-cell phenotyping revealed increased frequencies of leukocytes in the cerebrospinal fluid (CSF) of PACNS patients compared to patients with multiple sclerosis, ischemic stroke, and somatoform disorders (n = 18 per group). Changes in the intrathecal immune-cell profile were heterogeneous in PACNS. Read More

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http://dx.doi.org/10.1016/j.jneuroim.2018.06.004DOI Listing
August 2018
50 Reads

Cerebrospinal Fluid Concentrations of Neuronal Proteins Are Reduced in Primary Angiitis of the Central Nervous System.

Front Neurol 2018 5;9:407. Epub 2018 Jun 5.

Department of Neurology, University of Münster, Münster, Germany.

Primary angiitis of the central nervous system (PACNS) is a rare autoimmune vasculitis limited to the CNS often causing substantial disability. Understanding of this disease is impaired by the lack of available biomaterial. Here, we collected cerebrospinal fluid (CSF) from patients with PACNS and matched controls and performed unbiased proteomics profiling using ion mobility mass spectrometry to identify novel disease mechanisms and candidate biomarkers. Read More

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http://dx.doi.org/10.3389/fneur.2018.00407DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5996103PMC
June 2018
12 Reads

CNS Vasculitis: an Approach to Differential Diagnosis and Management.

Curr Rheumatol Rep 2018 05 30;20(7):37. Epub 2018 May 30.

Cleveland Clinic Center for Vasculitis Care and Research, 9500 Euclid Ave, A50, Cleveland, OH, 44195, USA.

Purpose Of Review: The goal of this review is to provide an up-to-date approach to diagnosis and management of patients with central nervous system (CNS) vasculitis.

Recent Findings: Challenges in diagnosis of CNS vasculitis still exist due to the broad differential diagnosis and generally nonspecific initial clinical manifestations. Differentiation between primary angiitis of the CNS (PACNS) and secondary causes is important in guiding management. Read More

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http://dx.doi.org/10.1007/s11926-018-0747-zDOI Listing
May 2018
12 Reads

Behçet's Syndrome and Nervous System Involvement.

Curr Neurol Neurosci Rep 2018 05 23;18(7):35. Epub 2018 May 23.

Cerrahpaşa School of Medicine, Department of Neurology, Istanbul University, Istanbul, Turkey.

Purpose Of Review: Although Behçet's syndrome (BS) is classified as a rare disease in European countries and the USA, its neurologic involvement "neuro-Behçet's syndrome (NBS)" is commonly included in the differential diagnosis of many inflammatory and vascular central nervous system (CNS) disorders. Clinical and neuroimaging findings support two major forms of NBS: parenchymal NBS (p-NBS) and an extra-parenchymal form that presents with cerebral venous sinus thrombosis (CVST). The present review summarizes recent findings on the etiopathogenesis and clinico-radiological features of this disorder as well as its treatment options. Read More

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http://dx.doi.org/10.1007/s11910-018-0843-5DOI Listing
May 2018
16 Reads

The first case of bacillus Calmette-Guérin-induced small-vessel central nervous system vasculitis.

Clin Rheumatol 2018 Aug 9;37(8):2297-2302. Epub 2018 May 9.

Department of Rheumatology, Centre Intégré Universitaire de Santé et de Services Sociaux de l'Estrie, Centre Hospitalier Universitaire de Sherbrooke, 3001 12e Avenue Nord, Sherbrooke, QC, J1H 5N4, Canada.

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. Read More

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http://link.springer.com/10.1007/s10067-018-4136-9
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http://dx.doi.org/10.1007/s10067-018-4136-9DOI Listing
August 2018
21 Reads
1.770 Impact Factor

Pearls & Oy-sters: The critical role of histopathology in diagnosing cancer-associated necrotizing CNS vasculitis.

Neurology 2018 04;90(17):808-811

From Wake Forest University School of Medicine (J.S.); and the Departments of Neurology (J.T., R.E.S., A.G.), Pathology (R.M.), Radiology (C.G.), and Rheumatology (R.W.), Wake Forest Baptist Medical Center, Winston-Salem, NC.

Objective: To highlight the importance of a broad differential and histopathologic confirmation in patients with newly diagnosed cancer with brain lesions atypical for CNS metastasis.

Methods: We report 2 cases of biopsy-proven CNS vasculitis in patients undergoing treatment for a newly diagnosed nonmetastatic cancer. Comprehensive medical record review was performed to identify the clinical presentation, representative neuroimaging, histopathologic features, and response to treatment. Read More

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http://dx.doi.org/10.1212/WNL.0000000000005350DOI Listing
April 2018
9 Reads

Primary lymphomatoid granulomatosis in the central nervous system: A report of three cases.

Neuropathology 2018 Apr 10. Epub 2018 Apr 10.

Department of Pathology, Seoul National University, College of Medicine, Seoul, Korea.

Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder characterized by infiltration of Epstein-Barr virus (EBV)-positive large atypical B-cells in an angiocentric fashion in a mixed inflammatory background. The histologic spectrum of LYG ranges from reactive proliferation to diffuse large B-cell lymphoma according to the number of EBV+ B-cells. It is known that virtually all patients have pulmonary involvement, whereas primary LYG of the other organs has been rarely reported. Read More

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http://dx.doi.org/10.1111/neup.12467DOI Listing
April 2018
51 Reads

Tc-HMPAO brain SPECT in the monitoring of cerebral vasculitis therapy.

Rev Esp Med Nucl Imagen Mol 2018 Jul - Aug;37(4):211-217. Epub 2018 Mar 28.

Department of Radiological, Oncological and Anatomo-Pathological Sciences, "Sapienza" University of Rome, Roma, Italia.

Objective: The central nervous system (CNS) may be involved in a variety of inflammatory diseases of the blood vessels, generally known as vasculitis. The clinical diagnosis of such involvement in early stages is difficult, since a mild cognitive impairment can be the only symptom. It was hypothesized that brain-perfusion SPECT would be able to reveal CNS involvement and to monitor the course of the disease. Read More

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http://dx.doi.org/10.1016/j.remn.2017.10.009DOI Listing
April 2019
5 Reads

Erratum: Spinal cord inflammation in children with small vessel primary cns vasculitis.

Authors:

Paediatr Child Health 2017 Nov 13;22(8):509. Epub 2017 Sep 13.

[This corrects the article DOI: 10.1093/pch/pxx086.079. Read More

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http://dx.doi.org/10.1093/pch/pxx130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5804907PMC
November 2017
8 Reads

Primary central nervous system vasculitis associated with lymphoma.

Neurology 2018 03 2;90(10):e847-e855. Epub 2018 Feb 2.

From the Departments of Neurology (R.D.B.), Biomedical Statistics and Informatics (T.J.H.C.), Radiology (J.H.), Hematology (S.M.A.), Anatomic Pathology (C.G.), and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN; and Rheumatology Division (C.S.), Azienda USL-IRCCS di Reggio Emilia e Università di Modena e Reggio Emilia, Italy. Dr. Salvarani is currently a visiting clinician at the Department of Neurology, Mayo Clinic, Rochester, MN.

Objectives: To record the clinical findings, response to therapy, and course of patients with primary CNS vasculitis (PCNSV) associated with lymphoma.

Patients And Methods: We reviewed the histories of 936 patients with a diagnosis of any type of vasculitis and lymphoma who were seen at the Mayo Clinic over a 32-year period. Ten patients with both PCNSV and lymphoma were identified. Read More

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http://dx.doi.org/10.1212/WNL.0000000000005062DOI Listing
March 2018
23 Reads

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.

Authors:
Leila Chimelli

Dement Neuropsychol 2017 Oct-Dec;11(4):356-363

Instituto Estadual do Cérebro Paulo Niemeyer - Laboratory of Neuropathology and Universidade Federal do Rio de Janeiro - Pathology Department, Rio de Janeiro, RJ - Brazil.

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. Read More

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http://www.scielo.br/scielo.php?script=sci_arttext&pid=S
Publisher Site
http://dx.doi.org/10.1590/1980-57642016dn11-040004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5769993PMC
January 2018
9 Reads

Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies.

Ann Neurol 2018 02 15;83(2):283-294. Epub 2018 Feb 15.

Department of Neuroscience, Drug and Child Health, University of Florence, Florence, Italy.

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. In a multicenter study, we assessed the frequency of perivenular lesions in MS versus systemic autoimmune diseases with CNS involvement and primary angiitis of the CNS (PACNS). Read More

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http://dx.doi.org/10.1002/ana.25146DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5901412PMC
February 2018
97 Reads