Raras
Buscar doenças, sintomas, genes...
Angeíte primária do sistema nervoso central
ORPHA:140989CID-10 · I67.7CID-11 · 4A44.7DOENÇA RARA

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.

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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.

Publicações científicas
410 artigos
Último publicado: 2026

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: I67.7
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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

🧠
Neurológico
7 sintomas
👁️
Olhos
2 sintomas
🫃
Digestivo
1 sintomas

+ 20 sintomas em outras categorias

Características mais comuns

90%prev.
Vasculite cerebral
Muito frequente (99-80%)
55%prev.
Pleocitose do LCR
Frequente (79-30%)
55%prev.
Cefaleia
Frequente (79-30%)
55%prev.
Anormalidades multifocais da substância branca cerebral
Frequente (79-30%)
55%prev.
Paralisia
Frequente (79-30%)
55%prev.
Hemiparesia
Frequente (79-30%)
30sintomas
Muito frequente (1)
Frequente (12)
Ocasional (12)
Muito raro (5)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 30 características clínicas mais associadas, ordenadas por frequência.

Vasculite cerebralCerebral vasculitis
Muito frequente (99-80%)90%
Pleocitose do LCRCSF pleocytosis
Frequente (79-30%)55%
CefaleiaHeadache
Frequente (79-30%)55%
Anormalidades multifocais da substância branca cerebralMultifocal cerebral white matter abnormalities
Frequente (79-30%)55%
ParalisiaParalysis
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico410PubMed
Últimos 10 anos200publicações
Pico202436 papers
Linha do tempo
2026Hoje · 2026🧪 2009Primeiro ensaio clínico📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Angeíte primária do sistema nervoso central

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

Nenhum ensaio clínico registrado para esta condição.

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Publicações mais relevantes

Timeline de publicações
240 papers (10 anos)
#1

Early immunosuppressive treatment guided by vessel wall enhancement on MRI for primary angiitis of the central nervous system in childhood.

BMJ case reports2026 Jan 29

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.

#2

Application of High-Resolution Magnetic Resonance Vessel Wall Imaging in Childhood Primary Angiitis of the Central Nervous System.

Journal of child neurology2026 Feb

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.

#3

SLIPPERS Reconsidered: Clinical, Radiological, and Pathological Overlap with PACNS-A Case Report.

Reports (MDPI)2026 Jan 31

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.

#4

Primary angiitis of the central nervous system: predictors of stroke during immunosuppressant treatment.

Insights into imaging2026 Feb 17

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.

#5

Central nervous system vasculitis mimicking a brain tumor: A case report.

Radiology case reports2026 Mar

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

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📚 EuropePMC261 artigos no totalmostrando 195

2026

SLIPPERS Reconsidered: Clinical, Radiological, and Pathological Overlap with PACNS-A Case Report.

Reports (MDPI)
2026

Primary angiitis of the central nervous system: predictors of stroke during immunosuppressant treatment.

Insights into imaging
2026

Early immunosuppressive treatment guided by vessel wall enhancement on MRI for primary angiitis of the central nervous system in childhood.

BMJ case reports
2026

Central nervous system vasculitis mimicking a brain tumor: A case report.

Radiology case reports
2025

A 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 immunology
2025

Rapidly progressive glucocorticoid-resistant childhood-primary angiitis of the central nervous system positive for HLA-B51 and A26: a case report.

Modern rheumatology case reports
2026

Primary angiitis of the central nervous system.

Current opinion in immunology
2025

Tumefactive primary angiitis of the central nervous system mimicking glioblastoma: A case-based systematic review.

Surgical neurology international
2025

Differentiating primary angiitis of the central nervous system from paraneoplastic neurological syndrome.

BMJ case reports
2025

Size of affected vessels in primary angiitis of the CNS: associations with clinical features, medical management and functional outcomes.

Frontiers in neurology
2025

Moyamoya Syndrome or Moyamoya Disease? Primary CNS Vasculitis in a Child? The Importance of Nomenclature.

International journal of rheumatic diseases
2025

A Case of Amyloid Beta-Related Angiitis: Rare Inflammatory Vasculopathy of the Central Nervous System.

Cureus
2026

Unilateral Primary Angiitis of the Central Nervous System: A Rare and Under-Recognized Entity.

AJNR. American journal of neuroradiology
2025

Role of RNF213 in Guiding Treatment of Moyamoya Disease with Unusual Phenotypic Presentation.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2025

Neurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous System.

Cells
2026

Application of High-Resolution Magnetic Resonance Vessel Wall Imaging in Childhood Primary Angiitis of the Central Nervous System.

Journal of child neurology
2025

Disease Characteristics and Treatments Associated with Outcome in Primary Angiitis of the Central Nervous System-A Multicenter Cohort Study in 163 Patients.

Annals of neurology
2025

Clinical Reasoning: Acute and Progressive Cognitive Decline in a 38-Year-Old Man.

Neurology
2025

Biopsy-proven PACNS: results from the large, multicentre cohort of cerebral vasculitis patients.

Journal of neurology, neurosurgery, and psychiatry
2025

Status epilepticus complicating a hemorrhagic and ischemic stroke due to primary cerebral angiitis: A case report.

Radiology case reports
2025

Rituximab treatment in Chinese patients with primary angiitis of the central nervous system.

Frontiers in neurology
2025

Inflammatory disorders that affect the cerebral small vessels.

Chinese medical journal
2025

Primary Angiitis of the Central Nervous System and Its Mimics: A Pattern-Based Approach to Biopsies for Central Nervous System Vasculitis.

Archives of pathology &amp; laboratory medicine
2025

Integrated Metabolomics and Transcriptomics Analyses Reveal Metabolic Changes in Primary Angiitis of the Central Nervous System.

Journal of inflammation research
2025

Primary Angiitis of the Central Nervous System Masquerading as a Pontine Glioma.

Neurology India
2025

Magnetic resonance spectroscopy for discriminating primary angiitis of the central nervous system from gliomas and lymphomas.

Acta neurologica Belgica
2024

Mass-like presentation of primary angiitis of the central nervous system: A clinical note.

Current journal of neurology
2024

Rituximab-Associated Acute Ischemic Stroke in a Patient With Primary Angiitis of the Central Nervous System.

Cureus
2024

Myelin oligodendrocyte glycoprotein antibody-associated disease with histopathologic features of primary CNS angiitis without demyelination: Case report and literature review.

Journal of neuroimmunology
2024

Prognostic Factors and Outcome Measures After Rituximab Therapy in Central Nervous System Vasculitis: A Systematic Review.

Cureus
2024

Unlikely Impersonator of Primary Brain Tumors: Illustrative Case of Primary Angiitis of the Central Nervous System.

Stroke
2024

A Rare Case of Idiopathic Reversible Cerebral Vasoconstriction Syndrome.

The American journal of case reports
2024

[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
2024

[Inflammatory causes of stroke-Diagnostics and treatment].

Der Nervenarzt
2024

Diagnostic MRI Score to Differentiate Susac Syndrome from Primary Angiitis of the Central Nervous System and Multiple Sclerosis.

Annals of neurology
2024

Cerebral Amyloid Angiopathy-Related Inflammation and Biopsy-Positive Primary Angiitis of the CNS: A Comparative Study.

Neurology
2024

18 F-FDG Brain PET/MRI in Granulomatous Primary Angiitis of the Central Nervous System.

Clinical nuclear medicine
2024

Primary Angiitis of the CNS: Differences in the Profile Between Subtypes and Outcomes From an Indian Cohort.

Neurology(R) neuroimmunology &amp; neuroinflammation
2024

Temporal evolution of primary angiitis of the central nervous system (PACNS) on MRI following immunosuppressant treatment.

Insights into imaging
2024

Time-of-flight MRA of intracranial vessels at 7 T.

European radiology experimental
2024

Vasculitis in the Central Nervous System: Etiology, Characteristics, and Outcomes in a Large Single-Center Cohort.

The Neurohospitalist
2024

Cortical Microhemorrhage Presentation of Small Vessel Primary Angiitis of the Central Nervous System.

Annals of neurology
2024

Primary Angiitis of the Central Nervous System: Exclusion of Differentials and Long-Term Follow-Up.

Journal of the Korean Society of Radiology
2024

Reversible Cerebral Vasoconstriction Syndrome With Typical and Atypical Symptoms: A Case Series.

Cureus
2023

PRIMARY ANGIITIS OF THE CENTRAL NERVOUS SYSTEM - A DIAGNOSTIC CHALLENGE.

Acta clinica Croatica
2024

Tumor-like Lesions in Primary Angiitis of the Central Nervous System: The Role of Magnetic Resonance Imaging in Differential Diagnosis.

Diagnostics (Basel, Switzerland)
2024

Progressive leukoencephalopathy as a manifestation of primary angiitis of the central nervous system: case report and review of the literature.

Journal of neurology
2024

The Hemorrhagic Side of Primary Angiitis of the Central Nervous System (PACNS).

Biomedicines
2024

Ruptured Saccular Aneurysm Caused by Necrotizing Arteritis of the Polyarteritis Nodosa Type in Primary Angiitis of Central Nervous System.

International journal of surgical pathology
2024

Primary Angiitis of Central Nervous System related intracranial aneurysm with spontaneous occlusion after immunomodulatory treatment.

Journal of cerebrovascular and endovascular neurosurgery
2024

Teaching NeuroImage: Unilateral Primary Angiitis of the CNS.

Neurology
2023

Case report: A case of primary angiitis of the central nervous system: misdiagnosed for 3.5 years.

Frontiers in neurology
2023

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

Journal of the Korean Society of Radiology
2024

Primary Angiitis of the Central Nervous System with Pathological Findings of Eosinophilic Granulomatosis with Polyangiitis.

Internal medicine (Tokyo, Japan)
2024

Advancements in the promotion of pyranoanthocyanins formation in wine: A review of current research.

Food chemistry
2023

Primary Angiitis of the Central Nervous System in Adults: A Comprehensive Review of 76 Biopsy-Proven Case Reports.

Journal of inflammation research
2024

Pathology of Primary Angiitis of the Central Nervous System.

Neuroimaging clinics of North America
2024

Treatment of Primary Angiitis of the Central Nervous System.

Neuroimaging clinics of North America
2023

European Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS).

European stroke journal
2024

Reduced Likelihood of Infarction in Crescendo Transient Ischemic Attack Caused by Vasculitic Middle Cerebral Artery Stenosis.

Internal medicine (Tokyo, Japan)
2024

Comparison of patients with biopsy positive and negative primary angiitis of the central nervous system.

Rheumatology (Oxford, England)
2024

Primary angiitis of the CNS and ANCA-associated vasculitis: from pathology to treatment.

Rheumatology international
2023

Childhood primary angiitis of the central nervous system following COVID-19 vaccination (BNT162b2/Pfizer-BioNtech): A case report.

Human vaccines &amp; immunotherapeutics
2023

Focal CNS vasculitis masquerading as new-onset focal aware seizures.

BMJ case reports
2024

Rare primary angiitis of the central nervous system affecting the clivus: A case report.

International journal of rheumatic diseases
2023

Cerebral vasculitis as a complication of pneumococcal meningitis: A cohort study.

Infectious diseases now
2024

Primary angiitis of the central nervous system (PANCS): a case report.

Acta neurologica Belgica
2023

Posterior Reversible Leucoencephalopathy Syndrome: Case Series, Comments, and Diagnostic Dilemma.

Current neurology and neuroscience reports
2023

Central Nervous System Vasculitis: Primary Angiitis of the Central Nervous System and Central Nervous System Manifestations of Systemic Vasculitis.

Rheumatic diseases clinics of North America
2023

Primary angiitis of the central nervous system in a patient with transient episodes of headache and aphasia: A case report.

Modern rheumatology case reports
2023

Reversible cerebral vasoconstriction syndrome: A review of pathogenesis, clinical presentation, and treatment.

International journal of stroke : official journal of the International Stroke Society
2024

A case of unexpected death due to primary angiitis of the central nervous system.

Forensic science, medicine, and pathology
2022

Primary Angiitis of the Central Nervous System: A Terra Incognita.

Annals of Indian Academy of Neurology
2022

Primary Angiitis of the Central Nervous System: A Great Mimicker.

Annals of Indian Academy of Neurology
2022

Primary Angiitis of the Central Nervous System - Diagnosis and Management.

Annals of Indian Academy of Neurology
2023

Close, but no cigar: an unfortunate case of primary angiitis of the central nervous system.

Annals of medicine and surgery (2012)
2023

The value of convolutional neural networks-based deep learning model in differential diagnosis of space-occupying brain diseases.

Frontiers in neurology
2023

Small 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 neurology
2022

A Special Case of Relapsing-Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis.

Brain sciences
2022

Soluble TREM2 is a potential biomarker for the severity of primary angiitis of the CNS.

Frontiers in immunology
2022

Misdiagnosis of Susac syndrome as demyelinating disease and primary angiitis of the central nervous system: A case report.

Frontiers in neurology
2023

Formation of hydroxyphenyl-pyranoanthocyanins derived from cyanidin-3-O-glucoside and effects of high-pressure processing on the transformation efficiency.

Food chemistry
2023

Diagnosis and management of adult primary angiitis of the central nervous system: an international survey on current practices.

Journal of neurology
2023

Childhood 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 Society
2022

Primary angiitis of the central nervous system as a mimic of multiple sclerosis: A case report.

Journal of neuroimmunology
2023

RCVS: by clinicians for clinicians-a narrative review.

Journal of neurology
2023

Mimics of Pediatric Small Vessel Primary Angiitis of the Central Nervous System.

Annals of neurology
2023

Efficacy of Proanthocyanidins in Nonsurgical Periodontal Therapy.

International dental journal
2022

Outcomes 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 Association
2022

Diagnostic and therapeutic approach to adult central nervous system vasculitis.

Revue neurologique
2022

Renal Involvement of CD20-Negative Intravascular Large B Cell Lymphoma with Neurological Manifestations.

Case reports in nephrology
2022

Primary Angiitis of the Central Nervous System: An Uncommon Cause of Stroke in the Young.

Cureus
2023

A case of childhood unilateral relapsing primary angiitis of the central nervous system.

Neuropathology : official journal of the Japanese Society of Neuropathology
2022

Isolated arteritis misdiagnosed as bilateral orbital tumors in a patient with acute ischemic stroke.

Radiology case reports
2022

Vertebrobasilar circulation hemorrhages in childhood primary angiitis of the central nervous system.

Autopsy &amp; case reports
2022

Right 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)
2021

Pseudo-insular glioma syndrome: illustrative cases.

Journal of neurosurgery. Case lessons
2022

A 5-year-old child presenting with tumor-like primary angiitis of the central nervous system.

Pediatric investigation
2022

Primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome: analysis of the National Inpatient Sample.

Clinical rheumatology
2022

Amyloid-β related angiitis presenting as eosinophilic meningitis: a case report.

BMC neurology
2022

Case Report: Interest of Positron Emission Tomography in Pediatric Small Vessel Primary Angiitis of the Central Nervous System.

Frontiers in pediatrics
2022

Primary angiitis of central nervous system - A challenging diagnosis.

Journal of neuroimmunology
2022

Intra-arterial application of nimodipine in reversible cerebral vasoconstriction syndrome: a neuroradiological method to help differentiate from primary central nervous system vasculitis.

Neurological research and practice
2022

Angiography-negative childhood primary angiitis of the central nervous system diagnosed by open brain biopsy: a case report.

Encephalitis (Seoul, Korea)
2022

Lethal Pediatric Cerebral Vasculitis Triggered by Severe Acute Respiratory Syndrome Coronavirus 2.

Pediatric neurology
2021

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

Frontiers in pediatrics
2021

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

Neurology(R) neuroimmunology &amp; neuroinflammation
2021

Unilateral Primary Angiitis of the Central Nervous System.

Annals of neurology
2021

Strokelore: Angiographic Diagnosis of Primary Angiitis of the Central Nervous System.

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association
2021

Comparison of Thalamus and Basal Ganglia Signs Between Multiple Sclerosis and Primary Angiitis of the Central Nervous System: An Exploratory Study.

Frontiers in neurology
2021

[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 shinpo
2021

Elevated intracranial pressure requiring decompressive craniectomy in a child with progressive primary angiitis of the central nervous system: a case report.

Journal of medical case reports
2021

Novel 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 research
2021

Neurological complications in COVID-19 - a diagnostic challenge.

Journal of medicine and life
2022

Vessel Wall Enhancement in Unilateral Primary Angiitis of the Central Nervous System.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2021

Profiling Complement System Components in Primary CNS Vasculitis.

Cells
2021

Isolated sixth nerve palsy as an initial presentation of primary angiitis of the central nervous system.

Brain &amp; development
2021

Primary Angiitis of Central Nervous System-Tumor-Like Mass Lesions: Report of Six Biopsy Confirmed Cases.

Neurology India
2021

Primary central nervous system vasculitis - An update on diagnosis, differential diagnosis and treatment.

Journal of the neurological sciences
2021

Rituximab for refractory primary angiitis of the central nervous system: Experience in two patients.

Multiple sclerosis and related disorders
2021

The diagnostic contribution of intracranial vessel wall imaging in the differentiation of primary angiitis of the central nervous system from other intracranial vasculopathies.

Neuroradiology
2021

Reversible Cerebral Vasoconstriction Syndrome without Headache That Was Initially Suspected of Being Primary Angiitis of the Central Nervous System.

Internal medicine (Tokyo, Japan)
2021

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

Neurology(R) neuroimmunology &amp; neuroinflammation
2020

Creutzfeldt-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?

Viruses
2020

An Unusual Cause of Intracerebral Hemorrhage: Clinical Pearls Regarding Primary Angiitis of the Central Nervous System.

Neurological sciences and neurosurgery
2020

Need for Swift Diagnosis of Primary Angiitis of Central Nervous System: A Case With Focal Motor Seizures of Hand Progressing to Aphasia.

Cureus
2020

Vessel Wall Imaging in Thunderclap Headache: A Reversible Cerebral Vasoconstriction Syndrome (RCVS) Case.

Headache
2020

Retrospective 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 Association
2020

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

Clinical neurology and neurosurgery
2020

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

Best practice &amp; research. Clinical rheumatology
2021

Extended stereotactic brain biopsy in suspected primary central nervous system angiitis: good diagnostic accuracy and high safety.

Journal of neurology
2021

Tocilizumab-A New Therapeutic Option for Primary Angiitis of the Central Nervous System?

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2020

"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 Neurophysiology
2020

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

Frontiers in pediatrics
2022

Recurrent intracerebral hemorrhages due to central nervous system vasculitis: A neuropsychological case report.

The Clinical neuropsychologist
2020

Reversible Cerebral Vasoconstriction Syndrome: A Common Occurrence but Rare Diagnosis.

Cureus
2020

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

Cureus
2020

IVIG in childhood primary angiitis of the central nervous system: A case report.

Brain &amp; development
2021

Heritable and non-heritable uncommon causes of stroke.

Journal of neurology
2020

Circulating Endothelial Cells as Promising Biomarkers in the Differential Diagnosis of Primary Angiitis of the Central Nervous System.

Frontiers in neurology
2020

Intracranial vessel wall imaging: applications, interpretation, and pitfalls.

Clinical radiology
2020

Intracranial Vessel Wall MRI in Cryptogenic Stroke and Intracranial Vasculitis.

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association
2020

Reversible Cerebral Vasoconstriction Syndrome: A Severe Neurological Complication in Postpartum Period.

Neurology India
2020

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

Journal of neuroimmunology
2020

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

Neurology
2020

Recurrent intracranial hemorrhage and cerebral venous sinus thrombosis: an atypical case of Neuro-Behçet's Syndrome.

Clinical neurology and neurosurgery
2020

Initial 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 imaging
2019

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

Acta neurologica Taiwanica
2019

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

Scientific reports
2019

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

Frontiers in neurology
2019

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

Case reports in neurological medicine
2020

Neuroimaging of Multiple Sclerosis Mimics.

Neurologic clinics
2019

Primary Angiitis of the Central Nervous System Presenting with Microhemorrhages on Gradient Echo Imaging.

Neurology India
2019

Ataxia as the main manifestation of tumor-like primary angiitis of the central nervous system: a case report and literature review.

BMC medical imaging
2020

Subacute cognitive impairment as clinical presentation of primary angiitis of the central nervous system.

Medicina clinica
2020

Childhood Small Vessel Primary Angiitis of the Central Nervous System: A Treatable Cause of Super-refractory Status Epilepticus.

Journal of child neurology
2019

[A Case of Cerebral Amyloid Angiopathy-related Subarachnoid Hemorrhage with Parkinson Disease Dementia].

No shinkei geka. Neurological surgery
2019

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

Neurology(R) neuroimmunology &amp; neuroinflammation
2019

Primary angiitis of the central nervous system (PACNS) - a rare and serious, but treatable entity.

Neurology India
2019

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

Neurology
2019

Biomarkers in Vasculitides of the Nervous System.

Frontiers in neurology
2019

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

Frontiers in neurology
2019

CSF Neurofilament light chain level predicts axonal damage in cerebral vasculitis.

Annals of clinical and translational neurology
2019

[Primary angiitis of the central nervous system with cerebral infarction and spinal hemorrhage].

Rinsho shinkeigaku = Clinical neurology
2018

Multiple 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 journal
2019

Biopsy-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 Australasia
2019

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

Journal of neurology
2019

Leading symptoms in cerebrovascular diseases: what about headache?

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2019

Primary central nervous system vasculitis in India - need for a multicenter prospective cohort study.

Neurology India
2019

Primary angiitis of the central nervous system - An Indian experience.

Neurology India
2019

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

Neurology India
2019

Long-term outcomes of patients with primary angiitis of the central nervous system.

Clinical and experimental rheumatology
2018

Partial Seizures as the First Manifestation of Primary Angiitis of the Central Nervous System.

European journal of case reports in internal medicine
2019

Atypical presentation of fulminant primary central nervous system angiitis.

Journal of neuroimmunology
2019

RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome.

Neurology
2019

Study 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 Association
2019

A 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 Neurosurgery
2019

Recurrent arterial ischemic stroke with good response to mycophenolate mofetil.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
2019

A 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 Neurophysiology
2018

Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system.

Journal of the neurological sciences
2018

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

Cureus
2018

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

Neurology
2018

Clinical, imaging, and histological presentations and outcomes of stroke related to sarcoidosis.

Journal of neurology
2018

Primary Angiitis of the Central Nervous System Presenting With Headache and Ataxia.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2018

Primary angiitis of the central nervous system: diagnosis and treatment.

Therapeutic advances in neurological disorders
2018

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

Rinsho shinkeigaku = Clinical neurology
2018

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

Journal of neuroimmunology
2018

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

Frontiers in neurology
2018

Rituximab treatment in primary angiitis of the central nervous system.

Internal medicine journal
2018

CNS Vasculitis: an Approach to Differential Diagnosis and Management.

Current rheumatology reports
2018

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

Clinical rheumatology
2018

Cerebrospinal fluid findings in reversible cerebral vasoconstriction syndrome: a way to differentiate from cerebral vasculitis?

Clinical and experimental immunology
2021

Primary angiitis of the central nervous system mimicking a cerebellar tumor.

British journal of neurosurgery
2018

[Small vessel-childhood primary angiitis of the central nervous system: a case report and literature review].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2018

Fatal reversible cerebral vasoconstriction syndrome.

Journal of the neurological sciences
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Doenças relacionadas

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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.

  1. Early immunosuppressive treatment guided by vessel wall enhancement on MRI for primary angiitis of the central nervous system in childhood.
    BMJ case reports· 2026· PMID 41611325mais citado
  2. Application of High-Resolution Magnetic Resonance Vessel Wall Imaging in Childhood Primary Angiitis of the Central Nervous System.
    Journal of child neurology· 2026· PMID 40589397mais citado
  3. SLIPPERS Reconsidered: Clinical, Radiological, and Pathological Overlap with PACNS-A Case Report.
    Reports (MDPI)· 2026· PMID 41718297mais citado
  4. Primary angiitis of the central nervous system: predictors of stroke during immunosuppressant treatment.
    Insights into imaging· 2026· PMID 41701418mais citado
  5. Central nervous system vasculitis mimicking a brain tumor: A case report.
    Radiology case reports· 2026· PMID 41503114mais citado
  6. Overview of Primary Angiitis of the Central Nervous System: Current Insights.
    Arch Intern Med Res· 2026· PMID 41947889recente
  7. Primary Angiitis of the Central Nervous System Mimicking a Cardioembolism: Posterior Circulation Infarction With Subarachnoid Hemorrhage and Obstructive Hydrocephalus.
    Cureus· 2026· PMID 41939632recente
  8. Reversible cerebral vasoconstriction syndrome: A narrative review.
    Headache· 2026· PMID 41902495recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:140989(Orphanet)
  2. MONDO:0015374(MONDO)
  3. GARD:8703(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. 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.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Angeíte primária do sistema nervoso central
Compêndio · Raras BR

Angeíte primária do sistema nervoso central

ORPHA:140989 · MONDO:0015374
Prevalência
Unknown
Herança
Not applicable
CID-10
I67.7 · Arterite cerebral não classificada em outra parte
CID-11
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0751878
EuropePMC
Wikidata
Wikipedia
Papers 10a
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