354 results match your criteria Primary Angiitis of the CNS

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 May 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 2021 May 3. 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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Primary Central Nervous System Vasculitis as an Unusual Cause of Intracerebral Hemorrhage: A Case Report.

Cureus 2021 Mar 12;13(3):e13847. Epub 2021 Mar 12.

Internal Medicine/Nephrology, McLaren Health Care, Flint/Michigan State University, Flint, USA.

A 64-year-old male with a history of transverse myelitis presented to the hospital with a decreased level of consciousness of one day's duration. CT of the head revealed intracranial hemorrhage measuring 2 x 1.2 cm in the right temporal lobe and multiple small hemorrhages in the left hemisphere, suggestive of vasculitis. Read More

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Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey.

Front Pediatr 2021 26;9:654537. Epub 2021 Mar 26.

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

Childhood Primary Angiitis of Central Nervous System (cPACNS) is rare, but can cause significant damage and result in disability or even death. Because of its rarity, the sometimes acute and variable presentation, limited awareness, and the absence of widely accepted diagnostic and therapeutic standards, cPACNS is a diagnostic and therapeutic challenge. Three subcategories of cPACNS exist, including angiography-positive non-progressive p-cPACNS, angiography-positive progressive p-cPACNS which both affects the medium to large vessels, and angiography-negative small vessel sv-cPACNS. Read More

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Brain Vessel Wall Contrast Enhancement Without Arterial Stenosis: Probable Primary CNS Vasculitis.

Neurol Clin Pract 2021 Apr 5;11(2):e193-e195. Epub 2020 Mar 5.

Department of Neurology (CG, MM, VL), University Hospital of Toulouse, France; ToNIC (CG, FB, VL), Toulouse NeuroImaging Center, University of Toulouse, Inserm, UPS; and Department of Neuroradiology (JD, FB), University Hospital of Toulouse, France.

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CNS involvement in systemic vasculitides.

Neil Scolding

J Neurol Sci 2021 05 27;424:117423. Epub 2021 Mar 27.

Burden Professor of Clinical Neurosciences, University of Bristol Institute of Clinical Neurosciences, UK, Gulu University Faculty of Medicine, Uganda. Electronic address:

Both the CNS and the PNS can be involved in almost all of the vasculitides - including the primary systemic vasculitic disorders, such as microscopic polyangiitis and polyarteritis nodosa, and in non-vasculitic systemic disorders, such as systemic lupus erythematosis and sarcoidosis. The latter diseases also include infections and toxininduced disorders - particularly drugs of abuse such as cocaine and amphetamines. Here we will summarise the spectrum of these disorders as they affect the CNS, concentrating in particular on their distinguishing clinical and investigational features. Read More

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Cerebral amyloid angiopathy and related inflammatory disorders.

B K Chwalisz

J Neurol Sci 2021 05 27;424:117425. Epub 2021 Mar 27.

Department of Neurology, Massachusetts General Hospital/Harvard Medical School, 15 Parkman Street, Suite 835, Boston, MA 02114, USA; Division of Neuro-Ophthalmology, Department of Ophthalmology, Massachusetts Eye & Ear Infirmary/Harvard Medical School, Boston, MA, USA. Electronic address:

Inflammatory cerebral amyloid angiopathy is a largely reversible inflammatory vasculopathy that develops in an acute or subacute fashion in reaction to amyloid protein deposition in the central nervous system blood vessels. There are two recognized pathologically characterized variants: cerebral amyloid angiopathy-related inflammation (CAAri) and A beta-related angiitis (ABRA). Both variants produce a clinical picture that resembles primary angiitis of the CNS but is distinguished by a characteristic radiologic appearance. Read More

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Unilateral Relapsing Primary Angiitis of the CNS: An Entity Suggesting Differences in the Immune Response Between the Cerebral Hemispheres.

Neurol Neuroimmunol Neuroinflamm 2021 03 5;8(2). Epub 2021 Jan 5.

From the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa.

Objective: To determine whether studying patients with strictly unilateral relapsing primary angiitis of the CNS (UR-PACNS) can support hemispheric differences in immune response mechanisms, we reviewed characteristics of a group of such patients.

Methods: We surveiled our institution for patients with UR-PACNS, after characterizing one such case. We defined UR-PACNS as PACNS with clinical and radiographic relapses strictly recurring in 1 brain hemisphere, with or without hemiatrophy. Read More

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Seizures in steroid-responsive encephalopathy.

Neurol Sci 2021 Feb 21;42(2):521-530. Epub 2020 Nov 21.

Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing Key Laboratory of Neurology, 1Youyi Road, Chongqing, 400016, China.

Steroid-responsive encephalopathy is a general term for diseases that are characterized by diffuse brain injury and respond well to corticosteroids or immunosuppressive agents, including Hashimoto's encephalopathy (HE), limbic encephalitis (LE), systemic lupus erythematosus encephalopathy (SLEE), antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitis encephalopathy (AASV), viral encephalitis (VE), and primary central nervous system lymphoma (PCNSL). Epilepsy and status epilepticus are the main manifestations of steroid-responsive encephalopathy. The spectrum of "autoimmune epilepsy" diseases, which has been approved by the epilepsy diagnostic recommendations of the International Antiepileptic League, is characterized by a high prevalence of epilepsy in central nervous system (CNS) autoimmune diseases and a variety of neuron-specific autoantibodies. Read More

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February 2021

Primary CNS vasculitis: A systematic review on clinical characteristics associated with abnormal biopsy and angiography.

Autoimmun Rev 2021 Jan 13;20(1):102714. Epub 2020 Nov 13.

Department of Clinical Neurological Sciences, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada. Electronic address:

Background: Primary Central Nervous System Vasculitis (PCNSV) remains a diagnostic challenge due to its variable and non-specific clinical manifestations. In part, the clinical heterogeneity of PCNSV may be a consequence of the modalities used for diagnosis; accordingly, there may be different subtypes of PCNSV based on whether the diagnosis was attained by biopsy or cerebral angiography.

Objective: To examine the frequency of symptoms, laboratory, and radiological features associated with PCNSV, and to identify distinct clinical features between biopsy and angiography defined PCNSV. Read More

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January 2021

Mimics of vasculitis.

Rheumatology (Oxford) 2021 01;60(1):34-47

Division of Rheumatology, University of California, Los Angeles, Los Angeles, CA, USA.

While prompt diagnosis of vasculitis is important, recognition of vasculitis mimics is equally essential. As in the case of vasculitis, an approach to mimics based on the anatomic size of vessels can be useful. Infections can mimic vasculitis of any vessel size, including the formation of aneurysms and induction of ANCAs. Read More

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January 2021

Characteristics of mass lesion presentation of primary angiitis of the central nervous system: A single center 11-year retrospective case-series study.

Clin Neurol Neurosurg 2020 12 8;199:106297. Epub 2020 Oct 8.

Department of Neurology, Sixth Medical Center of Chinese PLA General Hospital, Beijing, China. Electronic address:

Objective: Mass lesion presentation of primary angiitis of the central nervous system (ML-PACNS) is a special subtype of primary central nervous system vasculitis, which is difficult to be differentiated from other space-occupying disorders. We aimed to summarize our experience and improve diagnostic techniques of ML-PACNS.

Patients And Methods: Case records for 16 patients treated in the Department of Neurology of Six Medical Center of Chinese PLA General Hospital from December 2008 to November 2019 were examined. Read More

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December 2020

Tumefactive Primary Central Nervous System Vasculitis: Imaging Findings of a Rare and Underrecognized Neuroinflammatory Disease.

AJNR Am J Neuroradiol 2020 11 3;41(11):2075-2081. Epub 2020 Sep 3.

Radiology and Imaging Sciences (A.G.O.), University of Utah, Salt Lake City, Utah.

Primary central nervous system vasculitis (PCNSV) is a poorly understood neuroinflammatory disease of the CNS affecting the intracranial vasculature. Although PCNSV classically manifests as a multifocal beaded narrowing of the intracranial vessels, some patients may not have angiographic abnormalities. A rare subset of patients with PCNSV present with masslike brain lesions mimicking a neoplasm. Read More

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November 2020

CNS vasculopathies: Challenging mimickers of primary angiitis of the central nervous system.

Best Pract Res Clin Rheumatol 2020 08 25;34(4):101569. Epub 2020 Aug 25.

200 Hawkins Drive, Iowa City, IA, 52242, USA. Electronic address:

Primary angiitis of the central nervous system (CNS) is an inflammatory vasculopathy affecting the brain and spinal cord. It is a difficult diagnosis to make because of its insidious nonspecific course and its multiple mimics. This review identifies and discusses some noninfectious mimickers of primary CNS angiitis, including: reversible cerebral vasoconstriction syndrome, Sneddon's Syndrome, amyloid-beta-related angiopathy, Susac Syndrome, and neurosarcoidosis. Read More

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"When should primary angiitis of the central nervous system (PACNS) be suspected?": literature review and proposal of a preliminary screening algorithm.

Neurol Sci 2020 Nov 10;41(11):3135-3148. Epub 2020 Aug 10.

NEUROFARBA Department, Neuroscience Section, University of Florence, Largo Brambilla 3, 50134, Florence, Italy.

Background: Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect.

Objective: (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations. Read More

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November 2020

Clinical Perspective on Primary Angiitis of the Central Nervous System in Childhood (cPACNS).

Front Pediatr 2020 3;8:281. Epub 2020 Jul 3.

Abteilung Neuropädiatrie, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.

Non-arteriosclerotic arteriopathies have emerged as important underlying pathomechanism in pediatric arterial ischemic stroke (AIS). The pathogenesis and classification of cerebral arteriopathies in childhood are heterogeneous. Different classifications base on (i) the anatomic site; (ii) the distribution and size of the affected vessel; (iii) the time course, for example, transient vs. Read More

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Primary Angiitis of the Central Nervous System Presenting as a Cerebral Mass Lesion: A Case Report and Literature Review.

Cureus 2020 Jun 8;12(6):e8511. Epub 2020 Jun 8.

Neurosurgery, Kaiser Permanente, Los Angeles, USA.

Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis and is confined entirely to the central nervous system (CNS)without systemic involvement. We report a rare case of PACNS in a 39-year-old female with new onset seizures and a right frontal enhancing mass. Initially the patient was thought to have a high-grade glioma and thus underwent a right frontal craniotomy for resection of right frontal mass. Read More

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

Lancet Psychiatry 2020 10 25;7(10):875-882. 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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October 2020

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 09 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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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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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 04 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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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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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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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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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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February 2020