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Primary Angiitis of the CNS

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A Young Woman Presenting with Encephalopathy: A Case Report.

Mario Kofler, Stefan Kiechl, Raimund Helbok,

Neurocrit Care (Neurocritical care)
[2020, 32(2):630-632]

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Alemtuzumab-related eosinophilic central nervous system vasculitis.

Oliver A Leach, David A Hilton, William Adams, Seth Love, Agne Straukiene,

A 36-year-old woman with relapsing remitting multiple sclerosis (MS) presented with right-sided spasms, focal seizures and neuropsychiatric symptoms 10 months after her first course of alemtuzumab. Magnetic resonance imaging (MRI) brain imaging revealed multiple foci of T2 hyperintensity. Subsequent blood and cerebrospinal fluid (CSF) testing for progressive multifocal leukoencephalopathy (PML), vasculitis ... Read more >>

Mult. Scler. (Multiple sclerosis (Houndmills, Basingstoke, England))
[2020, :1352458519898113]

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

Zinovia-Maria Kefalopoulou, Stamatis-Nick Liossis, Theodora Sagona, Dimitra Veltsista, Petros Zampakis, Pantelis Kraniotis, Aikaterini Solomou, John Ellul, Elisabeth Chroni,

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 ... Read more >>

J. Neuroimmunol. (Journal of neuroimmunology)
[2020, 341:577190]

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Long-term remission, relapses and maintenance therapy in adult primary central nervous system vasculitis: A single-center 35-year experience.

Carlo Salvarani, Robert D Brown, Teresa J H Christianson, John Huston, Caterina Giannini, Gene G Hunder,

OBJECTIVES:To evaluate long-term treatment and outcomes of patients with primary central nervous system vasculitis (PCNSV). METHODS:In this cohort of 191 consecutive patients with PCNSV seen at Mayo Clinic, Rochester, MN, over 35 years with long-term follow-up we analyzed response to and duration of therapy, frequency of relapses, long-term remission, efficacy of ... Read more >>

Autoimmun Rev (Autoimmunity reviews)
[2020, 19(4):102497]

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[Case of isolated neurosarcoidosis requiring differentiation from tuberculous meningitis].

Ichiro Deguchi, Takashi Osada, Tomonari Suzuki, Shinya Tabata, Eiichi Arai, Akira Uchino, Takeshi Hayashi, Shinichi Takahashi, Suketaka Momoshima, Masaki Takao,

A 62-year-old woman was transported to our hospital for management of generalized clonic seizures. Cerebrospinal fluid examination showed an increased monocyte-dominant cell count, high protein concentration, and low glucose concentration that was 17% of the plasma glucose concentration. Contrast-enhanced cranial magnetic resonance imaging revealed diffuse leptomeningeal enhancement with multiple nodular ... Read more >>

Rinsho Shinkeigaku (Rinsho shinkeigaku = Clinical neurology)
[2020, 60(3):213-218]

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En mann i 60-årene med gangvansker, dysartri og lett kognitiv svikt.

Ludmila Nordaa, Elin Bjelland Forsaa, Audun Odland, Soffien Ajmi,

BACKGROUND:Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is an immune-mediated, treatable and inflammatory CNS disease first reported by Pittock et al. (2010). CASE PRESENTATION:We describe a 66-year-old man with previous history of diabetes, atrial fibrillation and hypertension, who was admitted to hospital with reduced general ... Read more >>

Tidsskr. Nor. Laegeforen. (Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke)
[2020, 140(4):]

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Primary CNS lymphoma initially diagnosed as vasculitis.

Lauren Feldman, Yuebing Li, Daniel Ontaneda,

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, ... Read more >>

Neurol Clin Pract (Neurology. Clinical practice)
[2020, 10(1):84-88]

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Central nervous system vasculitis: advances in diagnosis.

Rula A Hajj-Ali, Leonard H Calabrese,

PURPOSE OF REVIEW:The main purpose of this review is to present advances in diagnostics of central nervous system vasculitis (CNS-V). RECENT FINDINGS:Progress in molecular technologies and neuroimaging have added formidably to our knowledge of CNS-V. Next-generation sequencing has the promise to enhance our ability to diagnose, interrogate, and track infectious ... Read more >>

Curr Opin Rheumatol (Current opinion in rheumatology)
[2020, 32(1):41-46]

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Differentiation between neurosarcoidosis and primary central nervous system vasculitis based on demographic, cerebrospinal and imaging features.

Didem Saygin, Stephen Jones, Priya Sundaram, Leonard H Calabrese, William Messner, Jinny O Tavee, Rula A Hajj-Ali,

OBJECTIVES:Neurosarcoidosis (NS) and primary angiitis of central nervous system (PACNS) are inflammatory diseases affecting central nervous system, with overlapping clinical and pathological characteristics. Distinguishing these diseases is important given distinct therapeutic implications. In this study, we aimed to compare demographic, CSF and MRI characteristics between these two conditions. METHODS:All the ... Read more >>

Clin. Exp. Rheumatol. (Clinical and experimental rheumatology)
[2020, :]

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Associations and Outcomes Between Chronic Traumatic Encephalopathy and Vasculitis in Adult Patients.

Dennis Adjepong, Bilal Haider Malik,

Chronic traumatic encephalopathy (CTE) results from brain injuries and traumas due to accelerated impacts on the head. In severe cases, the diseases cause brain damage, given the head trauma. On the other hand, vasculitis occurs through antibodies that mistake protein vessels as foreign, hence fighting them and resulting in their damage. ... Read more >>

Cureus (Cureus)
[2020, 12(1):e6795]

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Central Nervous System Systemic Lupus Erythematosus (CNS-SLE) Vasculitis Mimicking Lewy Body Dementia: A Case Report Emphasizing the Role of Imaging With an Analysis of 33 Comparable Cases From the Scientific Literature.

Peter Abraham, Ian Neel, Steven Bishay, Daniel D Sewell,

INTRODUCTION:Neuropsychiatric symptoms occur in 30% to 40% of patients living with systemic lupus erythematosus (SLE). Brain imaging may play a pivotal role in determining the etiology as it did for the case presented here. METHODS:A new case of central nervous system (CNS) SLE is presented along with an analysis of ... Read more >>

J Geriatr Psychiatry Neurol (Journal of geriatric psychiatry and neurology)
[2020, :891988720901788]

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Grief-induced reversible cerebral vasoconstriction syndrome (RCVS).

Pooja Rao, Michael Francis McCullough, Jessica Stevens, Matthew A Edwardson,

Stress is under-recognised as a potential causative factor for reversible cerebral vasoconstriction syndrome (RCVS). Here we present a case of RCVS occurring during a time of extreme emotional duress. A 46-year-old female patient with medical history of bipolar disorder developed a severe headache during her father's funeral. The following day ... Read more >>

BMJ Case Rep (BMJ case reports)
[2020, 13(1):]

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Brain miliary enhancement.

Joseph C J Bot, Linda Mazzai, Rogier E Hagenbeek, Silvia Ingala, Bob van Oosten, Esther Sanchez-Aliaga, Frederik Barkhof,

PURPOSE:Miliary enhancement refers to the presence of multiple small, monomorphic, enhancing foci on T1-weighted post-contrast MRI images. In the absence of a clear clinical presentation, a broad differential diagnosis may result in invasive procedures and possibly brain biopsy for diagnostic purposes. METHODS:An extensive review of the literature is provided for ... Read more >>

Neuroradiology (Neuroradiology)
[2020, 62(3):283-300]

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Acute Susac Syndrome in a Recent User of Adulterated Cocaine: Levamisole as a Triggering Factor?

Klara De Baerdemaeker, Chiara Mabiglia, Philippe Hantson, Vincent Di Fazio, Thierry Duprez, Alexandra Kozyreff, Vincent van Pesch, Amina Sellimi,

Susac syndrome (SS) is a central nervous system vasculitis characterized by the clinical triad of encephalopathy, sensorineural hearing loss, and visual disturbance caused by branch retinal artery occlusion. It is considered as an inflammatory disorder, and an autoimmune etiology is suggested. A 29-year-old man with a history of recent cocaine ... Read more >>

Case Rep Neurol (Case reports in neurology)
[2020, 12(1):78-83]

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Management of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Insights from Multimodal Imaging with OCTA.

Mariana A. Oliveira, Jorge Simão, Amélia Martins, Cláudia Farinha,

A 28-year-old man presented to the emergency room with blurred vision in the right eye for two days. He reported a preceding flu-like illness one week earlier. His best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/25 in the left eye. There was no anterior chamber inflammation ... Read more >>

(Case Reports in Ophthalmological Medicine)
[2020, 2020:]

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

Jae W Song, Haochang Shou, Emmanuel C Obusez, Scott B Raymond, Samuel D Rafla, G Abbas Kharal, Pamela W Schaefer, Javier M Romero,

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.g., ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):19270]

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When a Stroke is not Just a Stroke: Escherichia Coli Meningitis with Ventriculitis and Vasculitis: A Case Report.

Barbara Ribeiro, Peter Bishop, Sima Jalili,

Introduction:Community-acquired Escherichia coli ventriculitis is considered a rare condition. Central nervous system (CNS) infection due to gram-negative bacilli is usually associated with previous neurosurgical interventions. The recent publication of cases of Escherichia coli meningitis and ventriculitis suggests its prevalence may be underestimated by the literature. Case presentation:A case of community-acquired ... Read more >>

J Crit Care Med (Targu Mures) (Journal of critical care medicine (Universitatea de Medicina si Farmacie din Targu-Mures))
[2020, 6(1):65-70]

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Acute Susac Syndrome in a Recent User of Adulterated Cocaine: Levamisole as a Triggering Factor?

Klara De Baerdemaeker, Chiara Mabiglia, Philippe Hantson, Vincent Di Fazio, Thierry Duprez, Alexandra Kozyreff, Vincent van Pesch, Amina Sellimi,

Susac syndrome (SS) is a central nervous system vasculitis characterized by the clinical triad of encephalopathy, sensorineural hearing loss, and visual disturbance caused by branch retinal artery occlusion. It is considered as an inflammatory disorder, and an autoimmune etiology is suggested. A 29-year-old man with a history of recent cocaine ... Read more >>

Case Rep Neurol (Case reports in neurology)
[2020, 12(1):78-83]

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Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient.

Tania Cellucci, Heather Van Mater, Francesc Graus, Eyal Muscal, William Gallentine, Marisa S Klein-Gitelman, Susanne M Benseler, Jennifer Frankovich, Mark P Gorman, Keith Van Haren, Josep Dalmau, Russell C Dale,

OBJECTIVE:Autoimmune encephalitis (AE) is an important and treatable cause of acute encephalitis. Diagnosis of AE in a developing child is challenging because of overlap in clinical presentations with other diseases and complexity of normal behavior changes. Existing diagnostic criteria for adult AE require modification to be applied to children, who ... Read more >>

Neurol Neuroimmunol Neuroinflamm (Neurology(R) neuroimmunology & neuroinflammation)
[2020, 7(2):]

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Vessel Wall Imaging of Cerebrovascular Disorders.

Kyle C Kern, David S Liebeskind,

PURPOSE OF REVIEW:High-resolution magnetic resonance vessel wall imaging (VWI) permits direct visualization of intracranial arterial wall pathology, providing diagnostic and prognostic information that is complementary to conventional imaging techniques. We highlight the most recent studies that have advanced the clinical application of VWI. RECENT FINDINGS:VWI aids in distinguishing and diagnosing ... Read more >>

Curr Treat Options Cardiovasc Med (Current treatment options in cardiovascular medicine)
[2019, 21(11):65]

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An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes.

Evan Berger, Ghassan Ghorayeb, Jeffery Hogg,

Purpose:To report an atypical case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with central nervous system (CNS) vasculitis and recurrent strokes. Observations:A 57 year-old female presented with APMPPE after a febrile illness and rash. She developed an acute infarct on magnetic resonance imaging. Computed tomography angiography of the cerebral ... Read more >>

Am J Ophthalmol Case Rep (American journal of ophthalmology case reports)
[2019, 16:100574]

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Development of Central Nervous System Vasculitis in a Patient with Waldenstrom Macroglobulinemia: A Rare Presentation with Poor Prognosis.

Tahir Muhammad Abdullah Khan, Yusra Ansari, Abdul Hasan Siddiqui, Saad Ali Ansari, Faraz Siddiqui,

Waldenstrom macroglobulinemia (WM) is a rare lymphoproliferative disorder characterized by the presence of monoclonal immunoglobulin M in serum. WM may present with neurologic complications involving the peripheral and central nervous systems (CNS) though CNS complications associated with WM are rare. We present a case of a 72-year-old male with an ... Read more >>

Cureus (Cureus)
[2019, 11(10):e6039]

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The diagnosis of primary central nervous system vasculitis.

Claire M Rice, Neil J Scolding,

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 ... Read more >>

Pract Neurol (Practical neurology)
[2020, 20(2):109-114]

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Elevated cerebrospinal fluid levels of total protein in patients with secondary central nervous system vasculitis and giant cell arteritis.

Yoshiyuki Abe, Mariko Harada, Kurisu Tada, Ken Yamaji, Naoto Tamura,

Objectives: Secondary central nervous system vasculitis (SCNSV) is an extremely rare, refractory, and fatal disease in patients with giant cell arteritis (GCA). We compared the characteristics of GCA patients with and without SCNSV. Methods: This retrospective, single-center, observational cohort study included 35 patients with GCA admitted to Juntendo University Hospital ... Read more >>

Mod Rheumatol (Modern rheumatology)
[2019, :1-6]

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Primary Angiitis of the Central Nervous System: From Psychiatry to Neurology.

Joel Leonardi, Christian Saleh, Phillip Jaszczuk, Kristine A Blackham, Sabrina Zechel, Stefanie Wilmes, Margret Hund-Georgiadis,

We present a case of a 54-year-old man with primary angiitis of the central nervous system (PACNS) who was initially admitted to a psychiatric clinic with a diagnosis of delirium. We discuss the difficulty in establishing the diagnosis of PACNS and provide the reader with some recommendations on how to ... Read more >>

Case Rep Neurol Med (Case reports in neurological medicine)
[2019, 2019:8074258]

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