380 results match your criteria Primary Angiitis of the CNS

Childhood Cerebral Vasculitis : A Multidisciplinary Approach.

Clin Neuroradiol 2022 Jun 24. Epub 2022 Jun 24.

Department of Medical Imaging, Children's Hospital of Eastern Ontario (CHEO), University of Ottawa, Ottawa, Ontario, Canada.

Cerebral vasculitis is increasingly recognized as a common cause of pediatric arterial stroke. It can present with focal neurological deficits, psychiatric manifestations, seizures, and encephalopathy. The etiopathogenesis of childhood cerebral vasculitis (CCV) is multifactorial, making an inclusive classification challenging. Read More

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[Primary CNS Vasculitis - An Overview].

Ther Umsch 2022 Jun;79(5):247-253

Universitätsklinik für Neurologie, Inselspital, Universitätsspital Bern und Universität Bern.

Primary CNS Vasculitis - An Overview Cerebral vasculitis, especially the primary vasculitis of the central nervous systems (primary CNS vasculitis), are rare inflammatory diseases of the small- and medium-sized vessels of the CNS. The pathogenesis of primary CNS vasculitis is unclear. Infectious triggers are hypothesized to induce an activation of the immune system with resulting inflammation of the blood vessels within the CNS. Read More

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A 14-year-old Girl with Right-Sided Weakness and Speech Difficulty.

Neurohospitalist 2022 Apr 16;12(2):408-412. Epub 2022 Feb 16.

Department of Pediatrics, Section of Child Neurology, University of Colorado School of Medicine, Aurora, CO, USA.

We herein report a case of Childhood Primary Central Nervous System (CNS) Angiitis. This case consisted of a 14-year-old girl who presented with right-sided weakness, aphasia, and a fever. A Magnetic Resonance Imaging of the brain showed multifocal areas of ischemia. Read More

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High-resolution Vessel Wall MRI: A New Armamentarium for Diagnosis of Primary Angiitis of CNS.

Vikas Bhatia

Ann Indian Acad Neurol 2021 Nov-Dec;24(6):845-846. Epub 2021 Oct 14.

Department of Radio-Diagnosis and Imaging, NIIR Section, PGIMER, Chandigarh, India.

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October 2021

Inpatient Management of Encephalopathy.

Cureus 2022 Feb 10;14(2):e22102. Epub 2022 Feb 10.

Neurology, University of Florida College of Medicine, Jacksonville, USA.

Unexplained encephalopathy is a common occurrence in tertiary care centers and neurologic disorders should be considered after ruling out the infectious, toxic and metabolic etiologies. Neuroimaging combined with a thorough history and examination is often helpful in ruling out stroke and fulminant demyelinating encephalopathies. Autoimmune encephalopathy should be suspected in any patient with unexplained acute or subacute onset encephalopathy or rapidly progressing dementia. Read More

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February 2022

Central nervous system vasculitis in a patient with axial spondyloarthritis treated with infliximab: A case report and literature review.

Eur J Rheumatol 2021 10;8(4):228-231

Clinic of Rheumatology, Naval Hospital of Athens, Athens, Greece.

Central nervous system (CNS) vasculitis is a rare form of vasculitis involving the blood vessels of the brain. It may be primary when it is confined to the CNS or secondary in the context of systemic inflammatory conditions such as systemic lupus erythematosus, rheumatoid arthritis, or infections. However, there is no known association with axial spondyloarthritis. Read More

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October 2021

A Case of Metastatic CNS Melanoma of Unknown Primary Presenting with Seizures.

Case Rep Med 2022 6;2022:3099750. Epub 2022 Jan 6.

Medical Specialists of the Palm Beaches Neurology, JFK Medical Center, Atlantis, FL, USA.

Seizures are a common occurrence. The goal of evaluating a seizure is to identify the etiology and to determine the likelihood of recurrence as well as guide management. We present a unique presentation of a 47-year-old female that presented with late onset seizures admitted due to status epilepticus. Read More

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January 2022

Case Report: Meningoencephalitis With Thrombotic Occlusive Vasculopathy in a Young EBV-Naïve Boy Is Associated With a Novel Mutation.

Front Immunol 2021 20;12:747738. Epub 2021 Dec 20.

Immunology Outpatient Clinic, Vienna, Austria.

X-linked lymphoproliferative disease (XLP1) is a combined immunodeficiency characterized by severe immune dysregulation caused by mutations in the gene. Loss or dysfunction of SH2D1A is associated with the inability in clearing Epstein-Barr-Virus (EBV) infections. Clinical manifestation is diverse and ranges from life-threatening hemophagocytic lymphohistiocytosis (HLH) and fulminant infectious mononucleosis (FIM) to lymphoma and antibody deficiency. Read More

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February 2022

TLR8/TLR7 dysregulation due to a novel TLR8 mutation causes severe autoimmune hemolytic anemia and autoinflammation in identical twins.

Am J Hematol 2022 03 28;97(3):338-351. Epub 2022 Jan 28.

CLIP - Childhood Leukaemia Investigation Prague, Department of Paediatric Haematology and Oncology, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic.

Our study presents a novel germline c.1715G>T (p.G572V) mutation in the gene encoding Toll-like receptor 8 (TLR8) causing an autoimmune and autoinflammatory disorder in a family with monozygotic male twins, who suffer from severe autoimmune hemolytic anemia worsening with infections, and autoinflammation presenting as fevers, enteritis, arthritis, and CNS vasculitis. Read More

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Primary CNS Vasculitis: Radiopathological Correlation.

Neurol India 2021 Nov-Dec;69(6):1619-1620

Department of Critical Care, KokilabenDhirubhai Ambani Hospital, Andheri West, Mumbai, Maharashtra, India.

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January 2022

Tocilizumab for severe refractory primary central nervous system vasculitis: A center experience.

Acta Neurol Scand 2022 Apr 5;145(4):479-483. Epub 2021 Dec 5.

Neurology Department, Centro Hospitalar Universitário de São João, Porto, Portugal.

Background: Primary Central Nervous System Vasculitis (PCNSV) is responsible for 3%-5% of strokes before age 50. It presents with clinical, radiological, and pathological variability. Optimal management is unknown given the absence of randomized clinical trials. Read More

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Update on treatment strategies for vasculitis affecting the central nervous system.

Drug Discov Today 2022 04 24;27(4):1142-1155. Epub 2021 Nov 24.

Institute of Neurointervention, Paracelsus Medical University, Salzburg, Austria; Department of Neurology, Christian Doppler University Hospital, Centre for Cognitive Neuroscience, Paracelsus Medical University Salzburg, Salzburg, Austria.

Vasculitis affecting the nervous system is a rare disease that can not only present with nonspecific initial symptoms, but also run a severe course without accurate treatment. Although improvements have been achieved, diagnosis of vasculitis remains challenging, because many classification criteria are unspecific or inconclusive with regard to central nervous system (CNS) manifestations. Currently, beside an isolated primary CNS vasculitis, several systemic types of vasculitis are known to affect the nervous system. Read More

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Focal status epilepticus may trigger relapse of primary angiitis of the CNS.

Epileptic Disord 2022 Feb;24(1):203-207

Saint-Luc University Hospital, Department of Neurology, Brussels, Belgium, Saint-Luc University Hospital, Centre for Refractory Epilepsy, Brussels, Belgium.

The role of neuroinflammation in epileptogenesis is extensively investigated, but short-term effects of seizures on established CNS pathologies are less studied and less predictable. We describe the case of a woman with previous recurrent episodes of focal cerebral haemorrhage of unknown cause who developed a pseudo-tumoural oedema triggered by provoked focal status epilepticus. A brain biopsy revealed that the underlying condition was primary angiitis of the CNS. Read More

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February 2022

High-Resolution Vessel Wall Imaging in Primary Angiitis of Central Nervous System.

Ann Indian Acad Neurol 2021 Jul-Aug;24(4):524-530. Epub 2021 May 21.

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

Background: High-resolution vessel wall imaging (HRVWI) can aid in differentiating the various intracranial vasculopathies, but has been sparingly used in the diagnosis of primary angiitis of central nervous system (PACNS). This study is aimed to describe the vessel wall imaging characteristics of PACNS.

Materials And Methods: Patients with confirmed diagnosis of PACNS according to the Calabrese and Mallek criteria who had abnormal HRVWI were included in this retrospective descriptive study. Read More

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Primary CNS vasculitis presenting with spinal cord involvement: a rare presentation.

Neurol Sci 2022 01 29;43(1):727-730. Epub 2021 Oct 29.

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.

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January 2022

Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey.

Front Pediatr 2021 12;9:756612. Epub 2021 Oct 12.

Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom.

Childhood primary angiitis of the Central Nervous System (cPACNS) is a rare autoimmune and inflammatory disease. It can result in significant neuronal damage, neurodevelopmental delay and potentially death. Childhood PACNS is divided into subcategories: angiography-positive p-cPACNS that affects medium and large vessels, and angiography-negative small vessel sv-cPACNS. Read More

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October 2021

Fused F-Choline PET/MRI: A potential non-invasive biomarker imaging tool in cerebral vasculitis.

Radiol Case Rep 2021 Dec 6;16(12):3794-3797. Epub 2021 Oct 6.

Cyclotron & Radiopharmaceuticals Department, King Faisal Specialist Hospital & Research Centre, MBC#28, P.O. Box 3354, Riyadh 11211, Saudi Arabia.

Primary CNS vasculitis is an inflammatory brain disease commonly misdiagnosed affecting the medium and small vessels of the CNS. Due to its broad and non-specific clinical and radiological manifestations; Its diagnosis remains challenging. New diagnostic tools and biomarkers which increase specificity and facilitate the diagnosis for patients with suspected vasculitis are highly desirable to enable physicians to start therapy that can alter its potential aggressive course like immunosuppressant. Read More

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December 2021

Mitochondrial Strokes: Diagnostic Challenges and Chameleons.

Genes (Basel) 2021 10 19;12(10). Epub 2021 Oct 19.

Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology and The National Hospital for Neurology and Neurosurgery, London WC1N 3BG, UK.

Mitochondrial stroke-like episodes (SLEs) are a hallmark of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). They should be suspected in anyone with an acute/subacute onset of focal neurological symptoms at any age and are usually driven by seizures. Suggestive features of an underlying mitochondrial pathology include evolving MRI lesions, often originating within the posterior brain regions, the presence of multisystemic involvement, including diabetes, deafness, or cardiomyopathy, and a positive family history. Read More

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October 2021

Primary Angiitis of the CNS: A Systematic Review and Meta-analysis.

Neurol Neuroimmunol Neuroinflamm 2021 11 18;8(6). Epub 2021 Oct 18.

From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Department of Neurology (T.R., S.M.), Heinrich-Heine-University, Düsseldorf, Germany.

Background And Objectives: To facilitate and improve the diagnostic and therapeutic process by systematically reviewing studies on patients with primary angiitis of the CNS (PACNS).

Methods: We searched PubMed, looking at the period between 1988 and February 2020. Studies with adult patients with PACNS were included. Read More

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November 2021

Primary phlebitis of central nervous system revealed by black-blood magnetic resonance imaging.

BMJ Case Rep 2021 Oct 13;14(10). Epub 2021 Oct 13.

Department of Neurology, Wakayama Medical University, Wakayama, Japan.

Primary phlebitis of the central nervous system (PPCNS) is a rare condition that might be a subset of primary angiitis of the CNS. In this case report, the patient was a 39-year-old man with a 2-week history of anterograde amnesia and abnormal behaviours. Black-blood MRI (BB-MRI) showed contrast enhancement of the left basilar vein and cerebral superficial veins. Read More

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October 2021

Headache in Multiple Sclerosis - Pharmacological Aspects.

Curr Pharm Des 2022 ;28(6):445-453

Department of Neurology, Neuroimmunological Section, University of Rostock, Rostock, Germany.

For decades, the headache was not considered a typical symptom of multiple sclerosis (MS) and was construed as a "red flag" for important differential diagnoses such as cerebral vasculitis. Meanwhile, several studies have demonstrated an increased prevalence of headaches in MS compared to the general population. This is due to the heterogeneity of headache genesis with frequent occurrence of both primary and secondary headaches in MS. Read More

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Primary central nervous system lymphoma in a patient with neuropsychiatric systemic lupus erythematosus receiving mycophenolate mofetil: A case report and literature review.

Mod Rheumatol Case Rep 2022 Jan;6(1):36-40

Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, Gunma, Japan.

A 41-year-old woman with a 14-month history of systemic lupus erythematosus (SLE) presented with headache, aphasia, and agraphia. A laboratory examination revealed mild proteinuria, hypocomplementemia, and elevated anti-double-stranded DNA antibody levels. A cerebrospinal fluid analysis demonstrated elevated protein and interleukin-6 levels. Read More

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January 2022

Pneumonia in Neurologic Disorders: Is Prophylaxis Necessary?

Neurol Clin Pract 2021 Jun;11(3):242-248

Department of Neurology, Neurological Institute, Cleveland Clinic, OH. Dr. Willis is now with Department of Neurology, University of Mississippi Medical Center, Jackson.

Background: The incidence of pneumonia (PJP) in patients with underlying neurologic conditions is not well established, and the necessity of PJP prophylaxis for immunocompromised patients with neurologic disorders is uncertain.

Methods: Single-center retrospective analysis of non-HIV PJP patients at a tertiary referral center from 2007 to 2016 to determine the incidence of PJP in patients with primary neurologic disorders.

Results: The study included 142 patients with PJP without HIV. Read More

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Inflammatory Diseases of the Central Nervous System.

Neurol Clin 2021 08 9;39(3):811-828. Epub 2021 Jun 9.

Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Co-appointment in Department of Neurology and Developmental Neuroscience, 6701 Fannin Street, 11th Floor, Houston, TX 77030, USA.

Pediatric neuroinflammatory conditions are a complex group of disorders with a wide range of clinical presentations. Patients can present with a combination of focal neurologic deficits, encephalopathy, seizures, movement disorders, or psychiatric manifestations. There are several ways that pediatric neuroinflammatory conditions can be classified, including clinical presentation, pathophysiologic mechanism, and imaging and laboratory findings. Read More

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Neurological complications in COVID-19 - a diagnostic challenge.

J Med Life 2021 Mar-Apr;14(2):216-224

Neurology Department, Cluj-Napoca County Emergency Hospital, Cluj-Napoca, Romania.

With the exponential growth of COVID-19 cases, the neurological complications reported during or after the infection became more common. There is limited knowledge regarding the pathophysiological mechanisms that are responsible for these complications. Recent data provides compelling evidence for the neurotropic nature of SARS-CoV-2, based on neurological manifestations reported during the current pandemic, as well as on previous experience with other coronaviruses. Read More

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Profiling Complement System Components in Primary CNS Vasculitis.

Cells 2021 05 8;10(5). Epub 2021 May 8.

Department of Neurology with Institute of Translational Neurology, University Hospital Münster, 48149 Münster, Germany.

Complement activation has been implicated in the pathogenesis of many vasculitic syndromes such as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Using an array-based multiplex system, we simultaneously quantified serum and CSF levels of activated and regulatory complement system proteins in patients with primary CNS vasculitis (PACNS; = 20) compared to patients with non-inflammatory conditions ( = 16). Compared to non-inflammatory controls, levels of C3a, C5a, and SC5b-9, indicative for general activation of the complement system, of C4a, specific for the activation of the classical pathway, Ba and Bb, reflective for alternative complement activation as well as concentrations of complement-inhibitory proteins factor H and factor I were unchanged in patients with PACNS. Read More

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[Primary Central Nervous System Vasculitis].

Yukio Takeshita

Brain Nerve 2021 May;73(5):511-515

Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.

Primary central nervous system (CNS) vasculitis is an uncommon disorder of unknown etiology that is restricted to the brain and spinal cord. Patients do not present with specific clinical features or a classical clinical course, and no blood or imaging investigations are available for diagnostic confirmation. Cerebral biopsy and angiography are the gold standards for diagnosis. Read More

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Aβ-related Angiitis (ABRA)-A Rare Paraneoplastic Cause of Cerebral Vasculitis in a Young Patient: A Pathway From Unspecific Neurological Symptoms to Final Diagnosis.

Neurologist 2021 May 5;26(3):103-107. Epub 2021 May 5.

Department of Neurology, City of Cologne Municipal Hospitals, Medical Center Cologne-Merheim.

Introduction: Aβ-related angiitis (ABRA) is a very rare disease entity with combined features of cerebral amyloid angiopathy and primary angiitis of the CNS. However, the pathogenesis has not been conclusively described yet. Interestingly though, a possible paraneoplastic origin has been reported in the past. Read More

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Vessel wall MR imaging of central nervous system vasculitis: a systematic review.

Neuroradiology 2022 Jan 3;64(1):43-58. Epub 2021 May 3.

Department of Radiology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA, 19104, USA.

Purpose: Beyond vessel wall enhancement, little is understood about vessel wall MR imaging (VW-MRI) features of vasculitis affecting the central nervous system (CNS). We reviewed vessel wall MR imaging patterns of inflammatory versus infectious vasculitis and also compared imaging patterns for intracranial versus extracranial arteries of the head and neck.

Methods: Studies reporting vasculitis of the CNS/head and neck and included MR imaging descriptions of vessel wall features were identified by searching PubMed, Scopus, Cochrane, Web of Science, and EMBASE up to June 10, 2020. Read More

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January 2022