Vasculite cerebral ou vasculite do sistema nervoso central é a inflamação dos vasos sanguíneos (vasculite) do cérebro e, em alguns casos, da espinal medula. A doença afeta todos os vasos sanguíneos, desde os vasos capilares e arteríolas até às artérias e veias. Quando a corrente sanguínea é reduzida ou bloqueada em vasos com vasculite, as partes do corpo irrigadas por esses vasos começam a sofrer necrose. A doença pode produzir uma grande diversidade de sintomas neurológicos, como dor de cabeça, erupções cutâneas, fadiga, dor nas articulações, dificuldade de locomoção ou coordenação motora, alterações sensoriais e alterações na percepção, pensamento ou comportamento, assim como um tumor cerebral que possa causar coma e hérnia cerebral.
Introdução
O que você precisa saber de cara
Doença inflamatória rara que afeta vasos sanguíneos do cérebro e medula. Causa sintomas neurológicos como cefaleia, paralisias, déficits visuais e cognitivos, com alterações no LCR.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 20 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 30 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Angeíte primária do sistema nervoso central
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Early immunosuppressive treatment guided by vessel wall enhancement on MRI for primary angiitis of the central nervous system in childhood.
We aimed to report a case presenting with abrupt onset of acute right hemiplegia and severe headache, which was diagnosed as primary angiitis of the central nervous system in childhood (cPACNS). MRI revealed an acute cerebral infarction and long-segment concentric vessel wall enhancement in the left middle cerebral artery, with the enhancement resolving within 26 days following prompt steroid therapy, earlier than in previous reports. Laboratory and cerebrospinal fluid findings were unremarkable, except for transient elevations in lactate and pyruvate levels. Other potential causes were excluded. Early immunosuppressive treatment with intravenous methylprednisolone, followed by oral steroids, caused gradual clinical improvement without cognitive decline. This case highlights the significance of contrast-enhanced MRI during the acute phase in patients presenting with cerebral infarction accompanied by headaches. In patients with cPACNS, early therapeutic intervention may cause the prompt resolution of contrast enhancement, reduce long-term sequelae and improve clinical outcomes.
Application of High-Resolution Magnetic Resonance Vessel Wall Imaging in Childhood Primary Angiitis of the Central Nervous System.
BackgroundChildhood primary angiitis of the central nervous system is a rare inflammatory disorder affecting the central nervous system in children. High-resolution magnetic resonance vessel wall imaging is less frequently used in the childhood primary angiitis of the central nervous system. This study aimed to investigate the value of high-resolution magnetic resonance vessel wall imaging in childhood primary angiitis of the central nervous system.MethodsWe retrospectively reviewed patients <18 years old who met the diagnostic criteria for primary angiitis of the central nervous system at Xiangya Hospital from January 2020 to April 2024. High-resolution magnetic resonance vessel wall imaging was performed to assess vessel wall enhancement, which was subsequently quantified for analysis.ResultA total of 6 patients were included in the study, 3 of whom were male, with a mean age of onset of 10.3 years. The most common presenting symptoms were headache/dizziness and hemiplegia. Ischemic brain lesions were caused by stenosis of vessels in the anterior circulation in 4 patients and in the posterior circulation in 2 patients. Intravenous methylprednisolone was the primary immunotherapy used and was administered to all patients. None experienced relapse. A total of 174 arteries were evaluated, of which 38 exhibited grade 1 enhancement and 31 exhibited grade 2 enhancement. Bilateral internal carotid artery wall enhancement was noted in all patients. Corresponding offending arteries were identified in each case, all of which showed predominant enhancement and varying degrees of stenosis. The vessel wall enhancement score at the initial assessment was 16.7 ± 2.8, which significantly decreased to 12.7 ± 2.4 at the final assessment (P = .012).ConclusionHigh-resolution magnetic resonance vessel wall imaging appears to be a potentially effective tool for both diagnosing childhood primary angiitis of the central nervous system and monitoring response to treatment.
SLIPPERS Reconsidered: Clinical, Radiological, and Pathological Overlap with PACNS-A Case Report.
Background and Clinical Significance: SLIPPERS syndrome (Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids) was first described in 2015 as a variant of CLIPPERS restricted to supratentorial regions. Only a few cases have been reported so far, and its distinction from primary angiitis of the central nervous system (PACNS) remains challenging, as both may present with overlapping clinical, radiological, and histopathological features. We report two patients initially diagnosed with SLIPPERS but finally fulfilling the diagnostic criteria for PACNS, highlighting the complexity of the differential diagnosis. Case Presentation: The first patient was a 49-year-old woman who presented with seizures, memory impairment, and facial neuralgia. MRI showed multiple cortico-subcortical and deep nodular lesions in the left hemisphere with gadolinium enhancement. Brain biopsy revealed a T-cell-predominant lymphocytic vascular infiltrate. She responded to corticosteroids but later relapsed, requiring methotrexate for long-term immunosuppression, with no further recurrences during seven years of follow-up. The second patient was a 64-year-old man with hypertension, dyslipidemia, and alcohol use who developed repeated focal-to-generalized seizures. MRI disclosed multifocal nodular gadolinium-enhancing right hemispheric lesions, with SWI microhemorrhages. Biopsy demonstrated transmural T-cell vasculitic infiltrates. He responded to corticosteroids and methotrexate, but radiological progression at 14 months prompted replacement with cyclophosphamide. Conclusions: There is a considerable clinical, radiological, and histological overlap between SLIPPERS and PACNS. Careful analysis of advanced MRI sequences, particularly angiographic and vessel-wall imaging studies, combined with meticulous histopathological analysis, is essential to avoid misdiagnosis. These similarities suggest that some cases attributed to SLIPPERS may, in fact, correspond to variants of PACNS.
Primary angiitis of the central nervous system: predictors of stroke during immunosuppressant treatment.
To evaluate predictors of ischemic stroke in patients with primary angiitis of the central nervous system after initiation of immunosuppressive therapy. This retrospective study included 204 MRI examinations of 23 patients with primary angiitis of the central nervous system, treated with immunosuppressive therapy between 2015 and 2020 at the University Hospital Bern and the Cantonal Hospital St. Gallen, Switzerland. Two senior neuroradiologists evaluated the MRI exams with regard to the occurrence and location of ischemic stroke and hemorrhage, as well as the following characteristics of inflamed vessels on 3D time-of-flight angiography and T1 dark-blood post contrast: signal intensity of vessel walls, length of enhancement, circular extent of enhancement, and stenosis. After matching ischemic strokes to their corresponding vessel, the temporal relationship of vessel alterations in accordance with therapy initiation and stroke onset was calculated. The majority (77.6%) of observed strokes were in the vascular territory of an inflamed vessel. A significant, non-linear temporal relationship between the timing of MRI and the initiation of immunosuppression was found. The highest predicted probability of ischemic stroke was observed between 10 and 20 days after the initiation of immunosuppressant therapy, reaching approximately 12%. Out of all evaluated vessel characteristics, a higher degree of stenosis (Estimate: 0.93, p = 0.006) and a higher circularity of enhancement (Estimate: 0.76, p = 0.01) were significantly associated with a higher likelihood of stroke. A better understanding of unfavorable constellations (critical timeframe, characteristic vessel wall changes) in patients treated for primary angiitis of the central nervous system may help to prevent secondary ischemic strokes. A better understanding of ischemic stroke predictors in patients treated for primary angiitis of the central nervous system may prompt closer monitoring or therapy adjustment. To evaluate risk factors for ischemic stroke in patients treated for primary angiitis of the central nervous system. Higher degree of stenosis and circular enhancement are associated with a higher likelihood of ischemic strokes, which typically occur between 10 and 20 days after therapy onset. Data obtained from this may prompt closer monitoring or therapy adjustment.
Central nervous system vasculitis mimicking a brain tumor: A case report.
Central nervous system (CNS) vasculitis is a rare inflammatory condition of blood vessels of the brain and spinal cord that can result in profound neurological symptoms mimicking many other pathologies. We present the case of a 36-year-old male who presented with subacute onset of progressive left-sided hemiparesis and refractory seizures. The diagnosis was challenging and required comprehensive review and integration of different imaging modalities, tissue sampling, and biochemical testing. This case highlights key imaging features which may aid in distinguishing central nervous system vasculitis from other etiologies such as malignancy.
Publicações recentes
Overview of Primary Angiitis of the Central Nervous System: Current Insights.
Primary Angiitis of the Central Nervous System Mimicking a Cardioembolism: Posterior Circulation Infarction With Subarachnoid Hemorrhage and Obstructive Hydrocephalus.
Reversible cerebral vasoconstriction syndrome: A narrative review.
SLIPPERS Reconsidered: Clinical, Radiological, and Pathological Overlap with PACNS-A Case Report.
Primary angiitis of the central nervous system: predictors of stroke during immunosuppressant treatment.
📚 EuropePMC261 artigos no totalmostrando 195
SLIPPERS Reconsidered: Clinical, Radiological, and Pathological Overlap with PACNS-A Case Report.
Reports (MDPI)Primary angiitis of the central nervous system: predictors of stroke during immunosuppressant treatment.
Insights into imagingEarly immunosuppressive treatment guided by vessel wall enhancement on MRI for primary angiitis of the central nervous system in childhood.
BMJ case reportsCentral nervous system vasculitis mimicking a brain tumor: A case report.
Radiology case reportsA comparative study of large-vessel and small-vessel primary angiitis of the central nervous system: insights from a Chinese single-center retrospective cohort.
Frontiers in immunologyRapidly progressive glucocorticoid-resistant childhood-primary angiitis of the central nervous system positive for HLA-B51 and A26: a case report.
Modern rheumatology case reportsPrimary angiitis of the central nervous system.
Current opinion in immunologyTumefactive primary angiitis of the central nervous system mimicking glioblastoma: A case-based systematic review.
Surgical neurology internationalDifferentiating primary angiitis of the central nervous system from paraneoplastic neurological syndrome.
BMJ case reportsSize of affected vessels in primary angiitis of the CNS: associations with clinical features, medical management and functional outcomes.
Frontiers in neurologyMoyamoya Syndrome or Moyamoya Disease? Primary CNS Vasculitis in a Child? The Importance of Nomenclature.
International journal of rheumatic diseasesA Case of Amyloid Beta-Related Angiitis: Rare Inflammatory Vasculopathy of the Central Nervous System.
CureusUnilateral Primary Angiitis of the Central Nervous System: A Rare and Under-Recognized Entity.
AJNR. American journal of neuroradiologyRole of RNF213 in Guiding Treatment of Moyamoya Disease with Unusual Phenotypic Presentation.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesNeurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous System.
CellsApplication of High-Resolution Magnetic Resonance Vessel Wall Imaging in Childhood Primary Angiitis of the Central Nervous System.
Journal of child neurologyDisease Characteristics and Treatments Associated with Outcome in Primary Angiitis of the Central Nervous System-A Multicenter Cohort Study in 163 Patients.
Annals of neurologyClinical Reasoning: Acute and Progressive Cognitive Decline in a 38-Year-Old Man.
NeurologyBiopsy-proven PACNS: results from the large, multicentre cohort of cerebral vasculitis patients.
Journal of neurology, neurosurgery, and psychiatryStatus epilepticus complicating a hemorrhagic and ischemic stroke due to primary cerebral angiitis: A case report.
Radiology case reportsRituximab treatment in Chinese patients with primary angiitis of the central nervous system.
Frontiers in neurologyInflammatory disorders that affect the cerebral small vessels.
Chinese medical journalPrimary Angiitis of the Central Nervous System and Its Mimics: A Pattern-Based Approach to Biopsies for Central Nervous System Vasculitis.
Archives of pathology & laboratory medicineIntegrated Metabolomics and Transcriptomics Analyses Reveal Metabolic Changes in Primary Angiitis of the Central Nervous System.
Journal of inflammation researchPrimary Angiitis of the Central Nervous System Masquerading as a Pontine Glioma.
Neurology IndiaMagnetic resonance spectroscopy for discriminating primary angiitis of the central nervous system from gliomas and lymphomas.
Acta neurologica BelgicaMass-like presentation of primary angiitis of the central nervous system: A clinical note.
Current journal of neurologyRituximab-Associated Acute Ischemic Stroke in a Patient With Primary Angiitis of the Central Nervous System.
CureusMyelin oligodendrocyte glycoprotein antibody-associated disease with histopathologic features of primary CNS angiitis without demyelination: Case report and literature review.
Journal of neuroimmunologyPrognostic Factors and Outcome Measures After Rituximab Therapy in Central Nervous System Vasculitis: A Systematic Review.
CureusUnlikely Impersonator of Primary Brain Tumors: Illustrative Case of Primary Angiitis of the Central Nervous System.
StrokeA Rare Case of Idiopathic Reversible Cerebral Vasoconstriction Syndrome.
The American journal of case reports[Clinical Features of 17 Patients With Primary Angiitis of the Central Nervous System Confirmed by Brain Biopsy].
Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae[Inflammatory causes of stroke-Diagnostics and treatment].
Der NervenarztDiagnostic MRI Score to Differentiate Susac Syndrome from Primary Angiitis of the Central Nervous System and Multiple Sclerosis.
Annals of neurologyCerebral Amyloid Angiopathy-Related Inflammation and Biopsy-Positive Primary Angiitis of the CNS: A Comparative Study.
Neurology18 F-FDG Brain PET/MRI in Granulomatous Primary Angiitis of the Central Nervous System.
Clinical nuclear medicinePrimary Angiitis of the CNS: Differences in the Profile Between Subtypes and Outcomes From an Indian Cohort.
Neurology(R) neuroimmunology & neuroinflammationTemporal evolution of primary angiitis of the central nervous system (PACNS) on MRI following immunosuppressant treatment.
Insights into imagingTime-of-flight MRA of intracranial vessels at 7 T.
European radiology experimentalVasculitis in the Central Nervous System: Etiology, Characteristics, and Outcomes in a Large Single-Center Cohort.
The NeurohospitalistCortical Microhemorrhage Presentation of Small Vessel Primary Angiitis of the Central Nervous System.
Annals of neurologyPrimary Angiitis of the Central Nervous System: Exclusion of Differentials and Long-Term Follow-Up.
Journal of the Korean Society of RadiologyReversible Cerebral Vasoconstriction Syndrome With Typical and Atypical Symptoms: A Case Series.
CureusPRIMARY ANGIITIS OF THE CENTRAL NERVOUS SYSTEM - A DIAGNOSTIC CHALLENGE.
Acta clinica CroaticaTumor-like Lesions in Primary Angiitis of the Central Nervous System: The Role of Magnetic Resonance Imaging in Differential Diagnosis.
Diagnostics (Basel, Switzerland)Progressive leukoencephalopathy as a manifestation of primary angiitis of the central nervous system: case report and review of the literature.
Journal of neurologyThe Hemorrhagic Side of Primary Angiitis of the Central Nervous System (PACNS).
BiomedicinesRuptured Saccular Aneurysm Caused by Necrotizing Arteritis of the Polyarteritis Nodosa Type in Primary Angiitis of Central Nervous System.
International journal of surgical pathologyPrimary Angiitis of Central Nervous System related intracranial aneurysm with spontaneous occlusion after immunomodulatory treatment.
Journal of cerebrovascular and endovascular neurosurgeryTeaching NeuroImage: Unilateral Primary Angiitis of the CNS.
NeurologyCase report: A case of primary angiitis of the central nervous system: misdiagnosed for 3.5 years.
Frontiers in neurologyNecrotizing Primary Angiitis of the Central Nervous System Mimicking Brain Abscess: A Case Report and Literature Review.
Journal of the Korean Society of RadiologyPrimary Angiitis of the Central Nervous System with Pathological Findings of Eosinophilic Granulomatosis with Polyangiitis.
Internal medicine (Tokyo, Japan)Advancements in the promotion of pyranoanthocyanins formation in wine: A review of current research.
Food chemistryPrimary Angiitis of the Central Nervous System in Adults: A Comprehensive Review of 76 Biopsy-Proven Case Reports.
Journal of inflammation researchPathology of Primary Angiitis of the Central Nervous System.
Neuroimaging clinics of North AmericaTreatment of Primary Angiitis of the Central Nervous System.
Neuroimaging clinics of North AmericaEuropean Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS).
European stroke journalReduced Likelihood of Infarction in Crescendo Transient Ischemic Attack Caused by Vasculitic Middle Cerebral Artery Stenosis.
Internal medicine (Tokyo, Japan)Comparison of patients with biopsy positive and negative primary angiitis of the central nervous system.
Rheumatology (Oxford, England)Primary angiitis of the CNS and ANCA-associated vasculitis: from pathology to treatment.
Rheumatology internationalChildhood primary angiitis of the central nervous system following COVID-19 vaccination (BNT162b2/Pfizer-BioNtech): A case report.
Human vaccines & immunotherapeuticsFocal CNS vasculitis masquerading as new-onset focal aware seizures.
BMJ case reportsRare primary angiitis of the central nervous system affecting the clivus: A case report.
International journal of rheumatic diseasesCerebral vasculitis as a complication of pneumococcal meningitis: A cohort study.
Infectious diseases nowPrimary angiitis of the central nervous system (PANCS): a case report.
Acta neurologica BelgicaPosterior Reversible Leucoencephalopathy Syndrome: Case Series, Comments, and Diagnostic Dilemma.
Current neurology and neuroscience reportsCentral Nervous System Vasculitis: Primary Angiitis of the Central Nervous System and Central Nervous System Manifestations of Systemic Vasculitis.
Rheumatic diseases clinics of North AmericaPrimary angiitis of the central nervous system in a patient with transient episodes of headache and aphasia: A case report.
Modern rheumatology case reportsReversible cerebral vasoconstriction syndrome: A review of pathogenesis, clinical presentation, and treatment.
International journal of stroke : official journal of the International Stroke SocietyA case of unexpected death due to primary angiitis of the central nervous system.
Forensic science, medicine, and pathologyPrimary Angiitis of the Central Nervous System: A Terra Incognita.
Annals of Indian Academy of NeurologyPrimary Angiitis of the Central Nervous System: A Great Mimicker.
Annals of Indian Academy of NeurologyPrimary Angiitis of the Central Nervous System - Diagnosis and Management.
Annals of Indian Academy of NeurologyClose, but no cigar: an unfortunate case of primary angiitis of the central nervous system.
Annals of medicine and surgery (2012)The value of convolutional neural networks-based deep learning model in differential diagnosis of space-occupying brain diseases.
Frontiers in neurologySmall vessel childhood primary angiitis of the central nervous system with positive anti-glial fibrillary acidic protein antibodies: a case report and review of literature.
BMC neurologyA Special Case of Relapsing-Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis.
Brain sciencesSoluble TREM2 is a potential biomarker for the severity of primary angiitis of the CNS.
Frontiers in immunologyMisdiagnosis of Susac syndrome as demyelinating disease and primary angiitis of the central nervous system: A case report.
Frontiers in neurologyFormation of hydroxyphenyl-pyranoanthocyanins derived from cyanidin-3-O-glucoside and effects of high-pressure processing on the transformation efficiency.
Food chemistryDiagnosis and management of adult primary angiitis of the central nervous system: an international survey on current practices.
Journal of neurologyChildhood Small-Vessel Primary Angiitis of the Central Nervous System: Overlap With MOG-Associated Disease.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyPrimary angiitis of the central nervous system as a mimic of multiple sclerosis: A case report.
Journal of neuroimmunologyRCVS: by clinicians for clinicians-a narrative review.
Journal of neurologyMimics of Pediatric Small Vessel Primary Angiitis of the Central Nervous System.
Annals of neurologyEfficacy of Proanthocyanidins in Nonsurgical Periodontal Therapy.
International dental journalOutcomes among patients with primary angiitis of the CNS: A Nationwide United States analysis.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke AssociationDiagnostic and therapeutic approach to adult central nervous system vasculitis.
Revue neurologiqueRenal Involvement of CD20-Negative Intravascular Large B Cell Lymphoma with Neurological Manifestations.
Case reports in nephrologyPrimary Angiitis of the Central Nervous System: An Uncommon Cause of Stroke in the Young.
CureusA case of childhood unilateral relapsing primary angiitis of the central nervous system.
Neuropathology : official journal of the Japanese Society of NeuropathologyIsolated arteritis misdiagnosed as bilateral orbital tumors in a patient with acute ischemic stroke.
Radiology case reportsVertebrobasilar circulation hemorrhages in childhood primary angiitis of the central nervous system.
Autopsy & case reportsRight arm weakness and mouth deviation as a presentation of Primary Angiitis of the Central Nervous System treated with rituximab: A case-report.
Annals of medicine and surgery (2012)Pseudo-insular glioma syndrome: illustrative cases.
Journal of neurosurgery. Case lessonsA 5-year-old child presenting with tumor-like primary angiitis of the central nervous system.
Pediatric investigationPrimary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome: analysis of the National Inpatient Sample.
Clinical rheumatologyAmyloid-β related angiitis presenting as eosinophilic meningitis: a case report.
BMC neurologyCase Report: Interest of Positron Emission Tomography in Pediatric Small Vessel Primary Angiitis of the Central Nervous System.
Frontiers in pediatricsPrimary angiitis of central nervous system - A challenging diagnosis.
Journal of neuroimmunologyIntra-arterial application of nimodipine in reversible cerebral vasoconstriction syndrome: a neuroradiological method to help differentiate from primary central nervous system vasculitis.
Neurological research and practiceAngiography-negative childhood primary angiitis of the central nervous system diagnosed by open brain biopsy: a case report.
Encephalitis (Seoul, Korea)Lethal Pediatric Cerebral Vasculitis Triggered by Severe Acute Respiratory Syndrome Coronavirus 2.
Pediatric neurologyDiagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey.
Frontiers in pediatricsPrimary Angiitis of the CNS: A Systematic Review and Meta-analysis.
Neurology(R) neuroimmunology & neuroinflammationUnilateral Primary Angiitis of the Central Nervous System.
Annals of neurologyStrokelore: Angiographic Diagnosis of Primary Angiitis of the Central Nervous System.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke AssociationComparison of Thalamus and Basal Ganglia Signs Between Multiple Sclerosis and Primary Angiitis of the Central Nervous System: An Exploratory Study.
Frontiers in neurology[Longitudinally Extensive Transverse Myelitis Caused by Granulomatous Primary Central Nervous System Vasculitis with Characteristic Brain Magnetic Resonance Image: A Case Report].
Brain and nerve = Shinkei kenkyu no shinpoElevated intracranial pressure requiring decompressive craniectomy in a child with progressive primary angiitis of the central nervous system: a case report.
Journal of medical case reportsNovel method using DW-MRI and ADC images to guide stereotactic biopsy for the diagnosis small primary angiitis of the central nervous system: a case report.
European journal of medical researchNeurological complications in COVID-19 - a diagnostic challenge.
Journal of medicine and lifeVessel Wall Enhancement in Unilateral Primary Angiitis of the Central Nervous System.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesProfiling Complement System Components in Primary CNS Vasculitis.
CellsIsolated sixth nerve palsy as an initial presentation of primary angiitis of the central nervous system.
Brain & developmentPrimary Angiitis of Central Nervous System-Tumor-Like Mass Lesions: Report of Six Biopsy Confirmed Cases.
Neurology IndiaPrimary central nervous system vasculitis - An update on diagnosis, differential diagnosis and treatment.
Journal of the neurological sciencesRituximab for refractory primary angiitis of the central nervous system: Experience in two patients.
Multiple sclerosis and related disordersThe diagnostic contribution of intracranial vessel wall imaging in the differentiation of primary angiitis of the central nervous system from other intracranial vasculopathies.
NeuroradiologyReversible Cerebral Vasoconstriction Syndrome without Headache That Was Initially Suspected of Being Primary Angiitis of the Central Nervous System.
Internal medicine (Tokyo, Japan)Unilateral Relapsing Primary Angiitis of the CNS: An Entity Suggesting Differences in the Immune Response Between the Cerebral Hemispheres.
Neurology(R) neuroimmunology & neuroinflammationCreutzfeldt-Jakob Disease with a Five-Year Clinical Course, Multicentric Cerebellar Prion Plaques and Prior History of Biopsy-Proven Primary Angiitis of the Central Nervous System: A Case for Iatrogenic Exposure?
VirusesAn Unusual Cause of Intracerebral Hemorrhage: Clinical Pearls Regarding Primary Angiitis of the Central Nervous System.
Neurological sciences and neurosurgeryNeed for Swift Diagnosis of Primary Angiitis of Central Nervous System: A Case With Focal Motor Seizures of Hand Progressing to Aphasia.
CureusVessel Wall Imaging in Thunderclap Headache: A Reversible Cerebral Vasoconstriction Syndrome (RCVS) Case.
HeadacheRetrospective Analysis of 28 Cases Confirmed for Primary Angiitis of the Central Nervous System by Biopsy.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke AssociationCharacteristics of mass lesion presentation of primary angiitis of the central nervous system: A single center 11-year retrospective case-series study.
Clinical neurology and neurosurgeryCNS vasculopathies: Challenging mimickers of primary angiitis of the central nervous system.
Best practice & research. Clinical rheumatologyExtended stereotactic brain biopsy in suspected primary central nervous system angiitis: good diagnostic accuracy and high safety.
Journal of neurologyTocilizumab-A New Therapeutic Option for Primary Angiitis of the Central Nervous System?
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases"When should primary angiitis of the central nervous system (PACNS) be suspected?": literature review and proposal of a preliminary screening algorithm.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyClinical Perspective on Primary Angiitis of the Central Nervous System in Childhood (cPACNS).
Frontiers in pediatricsRecurrent intracerebral hemorrhages due to central nervous system vasculitis: A neuropsychological case report.
The Clinical neuropsychologistReversible Cerebral Vasoconstriction Syndrome: A Common Occurrence but Rare Diagnosis.
CureusPrimary Angiitis of the Central Nervous System Presenting as a Cerebral Mass Lesion: A Case Report and Literature Review.
CureusIVIG in childhood primary angiitis of the central nervous system: A case report.
Brain & developmentHeritable and non-heritable uncommon causes of stroke.
Journal of neurologyCirculating Endothelial Cells as Promising Biomarkers in the Differential Diagnosis of Primary Angiitis of the Central Nervous System.
Frontiers in neurologyIntracranial vessel wall imaging: applications, interpretation, and pitfalls.
Clinical radiologyIntracranial Vessel Wall MRI in Cryptogenic Stroke and Intracranial Vasculitis.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke AssociationReversible Cerebral Vasoconstriction Syndrome: A Severe Neurological Complication in Postpartum Period.
Neurology IndiaAn ischemic stroke as the presenting manifestation of rapidly progressive primary angiitis of central nervous system in a 17-year-old boy.
Journal of neuroimmunologyPearls & Oy-sters: Primary angiitis of the CNS presenting with recurrent intracranial hemorrhage.
NeurologyRecurrent intracranial hemorrhage and cerebral venous sinus thrombosis: an atypical case of Neuro-Behçet's Syndrome.
Clinical neurology and neurosurgeryInitial experience with [18F]DPA-714 TSPO-PET to image inflammation in primary angiitis of the central nervous system.
European journal of nuclear medicine and molecular imagingCerebellar PACNS in an Elderly Patient Present as a Tumor-Like Mass Lesion: A case Report.
Acta neurologica TaiwanicaSpatial Distribution of Intracranial Vessel Wall Enhancement in Hypertension and Primary Angiitis of the CNS.
Scientific reportsPrimary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review.
Frontiers in neurologyPrimary Angiitis of the Central Nervous System: From Psychiatry to Neurology.
Case reports in neurological medicineNeuroimaging of Multiple Sclerosis Mimics.
Neurologic clinicsPrimary Angiitis of the Central Nervous System Presenting with Microhemorrhages on Gradient Echo Imaging.
Neurology IndiaAtaxia as the main manifestation of tumor-like primary angiitis of the central nervous system: a case report and literature review.
BMC medical imagingSubacute cognitive impairment as clinical presentation of primary angiitis of the central nervous system.
Medicina clinicaChildhood Small Vessel Primary Angiitis of the Central Nervous System: A Treatable Cause of Super-refractory Status Epilepticus.
Journal of child neurology[A Case of Cerebral Amyloid Angiopathy-related Subarachnoid Hemorrhage with Parkinson Disease Dementia].
No shinkei geka. Neurological surgeryStrategies for treatment of childhood primary angiitis of the central nervous system.
Neurology(R) neuroimmunology & neuroinflammationPrimary angiitis of the central nervous system (PACNS) - a rare and serious, but treatable entity.
Neurology IndiaExploratory proteomic analysis implicates the alternative complement cascade in primary CNS vasculitis.
NeurologyBiomarkers in Vasculitides of the Nervous System.
Frontiers in neurologyPrimary Angiitis of the Central Nervous System: New Potential Imaging Techniques and Biomarkers in Blood and Cerebrospinal Fluid.
Frontiers in neurologyCSF Neurofilament light chain level predicts axonal damage in cerebral vasculitis.
Annals of clinical and translational neurology[Primary angiitis of the central nervous system with cerebral infarction and spinal hemorrhage].
Rinsho shinkeigaku = Clinical neurologyMultiple subacute bilateral cerebral infarcts in a 9-year-old Nigerian male: a case of childhood primary angiitis of the central nervous system.
The Pan African medical journalBiopsy-proven primary angiitis of the central nervous system mimicking leukodystrophy: A case report and review of the literature.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaRelapse rates and long-term outcome in primary angiitis of the central nervous system.
Journal of neurologyLeading symptoms in cerebrovascular diseases: what about headache?
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyPrimary central nervous system vasculitis in India - need for a multicenter prospective cohort study.
Neurology IndiaPrimary angiitis of the central nervous system - An Indian experience.
Neurology IndiaPrimary angiitis of the central nervous system: Clinical profiles and outcomes of 45 patients.
Neurology IndiaLong-term outcomes of patients with primary angiitis of the central nervous system.
Clinical and experimental rheumatologyPartial Seizures as the First Manifestation of Primary Angiitis of the Central Nervous System.
European journal of case reports in internal medicineAtypical presentation of fulminant primary central nervous system angiitis.
Journal of neuroimmunologyRCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome.
NeurologyStudy on the Clinical, Imaging, and Pathological Characteristics of 18 Cases with Primary Central Nervous System Vasculitis.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke AssociationA case of central nervous system vasculitis presenting as a mass-like lesion.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryRecurrent arterial ischemic stroke with good response to mycophenolate mofetil.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyA case of primary angiitis of the central nervous system presenting with diffuse cerebral microbleeds and recurrent intracranial hemorrhage.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyClinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system.
Journal of the neurological sciencesIntracranial Atherosclerosis Versus Primary Angiitis of the Central Nervous System: a Case Report.
CureusPrimary angiitis of the CNS and reversible cerebral vasoconstriction syndrome: A comparative study.
NeurologyClinical, imaging, and histological presentations and outcomes of stroke related to sarcoidosis.
Journal of neurologyPrimary Angiitis of the Central Nervous System Presenting With Headache and Ataxia.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesPrimary angiitis of the central nervous system: diagnosis and treatment.
Therapeutic advances in neurological disorders[Misleading cerebral arterial wall gadolinium-enhancement in malignant lymphoma].
Rinsho shinkeigaku = Clinical neurologyImmune cell profiling in the cerebrospinal fluid of patients with primary angiitis of the central nervous system reflects the heterogeneity of the disease.
Journal of neuroimmunologyCerebrospinal Fluid Concentrations of Neuronal Proteins Are Reduced in Primary Angiitis of the Central Nervous System.
Frontiers in neurologyRituximab treatment in primary angiitis of the central nervous system.
Internal medicine journalCNS Vasculitis: an Approach to Differential Diagnosis and Management.
Current rheumatology reportsThe first case of bacillus Calmette-Guérin-induced small-vessel central nervous system vasculitis.
Clinical rheumatologyCerebrospinal fluid findings in reversible cerebral vasoconstriction syndrome: a way to differentiate from cerebral vasculitis?
Clinical and experimental immunologyPrimary angiitis of the central nervous system mimicking a cerebellar tumor.
British journal of neurosurgery[Small vessel-childhood primary angiitis of the central nervous system: a case report and literature review].
Zhonghua er ke za zhi = Chinese journal of pediatricsFatal reversible cerebral vasoconstriction syndrome.
Journal of the neurological sciencesAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Early immunosuppressive treatment guided by vessel wall enhancement on MRI for primary angiitis of the central nervous system in childhood.
- Application of High-Resolution Magnetic Resonance Vessel Wall Imaging in Childhood Primary Angiitis of the Central Nervous System.
- SLIPPERS Reconsidered: Clinical, Radiological, and Pathological Overlap with PACNS-A Case Report.
- Primary angiitis of the central nervous system: predictors of stroke during immunosuppressant treatment.
- Central nervous system vasculitis mimicking a brain tumor: A case report.
- Overview of Primary Angiitis of the Central Nervous System: Current Insights.
- Primary Angiitis of the Central Nervous System Mimicking a Cardioembolism: Posterior Circulation Infarction With Subarachnoid Hemorrhage and Obstructive Hydrocephalus.
- Reversible cerebral vasoconstriction syndrome: A narrative review.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:140989(Orphanet)
- MONDO:0015374(MONDO)
- GARD:8703(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q191557(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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