Raras
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Siderose superficial
ORPHA:247245CID-10 · I69.0DOENÇA RARA

A siderose superficial é uma doença neurológica rara caracterizada por perda auditiva neurossensorial progressiva, ataxia cerebelar, sinais piramidais e achados de neuroimagem revelando depósitos de hemossiderina nas leptomeninges espinhais e cranianas e na camada subpial. A doença progride lentamente e os pacientes podem apresentar comprometimento cognitivo leve, nistagmo, dismetria, espasticidade, disdiadococinesia, disartria, hiperreflexia e sinais de Babinski. Características adicionais relatadas incluem demência, incontinência urinária, anosmia, ageusia e anisocoria.

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Introdução

O que você precisa saber de cara

📋

A siderose superficial é uma doença neurológica rara caracterizada por perda auditiva neurossensorial progressiva, ataxia cerebelar, sinais piramidais e achados de neuroimagem revelando depósitos de hemossiderina nas leptomeninges espinhais e cranianas e na camada subpial. A doença progride lentamente e os pacientes podem apresentar comprometimento cognitivo leve, nistagmo, dismetria, espasticidade, disdiadococinesia, disartria, hiperreflexia e sinais de Babinski. Características adicionais relatadas incluem demência, incontinência urinária, anosmia, ageusia e anisocoria.

Pesquisas ativas
2 ensaios
8 total registrados no ClinicalTrials.gov
Publicações científicas
923 artigos
Último publicado: 2026 Apr 13

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
Casos conhecidos
300
pacientes catalogados
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: I69.0
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
11 sintomas
🦴
Ossos e articulações
2 sintomas
🫃
Digestivo
2 sintomas
🩸
Sangue
2 sintomas
👂
Ouvidos
2 sintomas
💪
Músculos
1 sintomas

+ 28 sintomas em outras categorias

Características mais comuns

90%prev.
Anormalidade do nervo vestibulococlear
Muito frequente (99-80%)
90%prev.
Ataxia
Muito frequente (99-80%)
90%prev.
Deficiência auditiva neurossensorial bilateral
Muito frequente (99-80%)
55%prev.
Morfologia anormal do corpo caloso
Frequente (79-30%)
55%prev.
Ataxia da marcha progressiva
Frequente (79-30%)
55%prev.
Atrofia cerebelar
Frequente (79-30%)
49sintomas
Muito frequente (3)
Frequente (7)
Ocasional (36)
Muito raro (3)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 49 características clínicas mais associadas, ordenadas por frequência.

Anormalidade do nervo vestibulococlearAbnormality of the vestibulocochlear nerve
Muito frequente (99-80%)90%
Ataxia
Muito frequente (99-80%)90%
Deficiência auditiva neurossensorial bilateralBilateral sensorineural hearing impairment
Muito frequente (99-80%)90%
Morfologia anormal do corpo calosoAbnormal corpus callosum morphology
Frequente (79-30%)55%
Ataxia da marcha progressivaProgressive gait ataxia
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico923PubMed
Últimos 10 anos200publicações
Pico202581 papers
Linha do tempo
2026Hoje · 2026🧪 2012Primeiro ensaio clínico📈 2025Ano 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

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico5
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 5 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Siderose superficial

🗺️

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

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

8 ensaios clínicos encontrados, 2 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
599 papers (10 anos)
#1

[Amyloid β-related angiitis (ABRA) with dural contrast enhancement].

Rinsho shinkeigaku = Clinical neurology2026 Mar 14

An 82-year-old woman presented with a rapidly progressive decline in cognitive function, which had previously been worsening over several months. Brain MRI revealed a lesion predominantly involving the leptomeninges of the right parietal lobe. Based on the presence of leptomeningeal-predominant lesions, cerebral microbleeds, and superficial siderosis, amyloid β-related angiitis (ABRA) was suspected. However, due to atypical findings such as dural enhancement, we performed a brain biopsy, leading to a definitive diagnosis of ABRA. Steroid therapy resulted in improvements in both clinical symptoms and imaging findings. Imaging findings in this case suggest that inflammation originating in the leptomeninges extended bidirectionally into both the brain parenchyma and the dura mater. This progression pattern is of particular interest for understanding the pathophysiology of ABRA and may offer a future possibility of avoiding brain biopsy by identifying characteristic imaging features.

#2

Quantitative evaluation of iron chelator effects on central motor and sensory tracts in superficial siderosis.

Frontiers in neurology2026

Superficial siderosis (SS) is a rare neurodegenerative disease characterized by hemosiderin deposition in the central nervous system, leading to progressive neuronal degeneration. Although myelopathy is a main clinical manifestation in SS, objective biomarkers for monitoring disease progression and treatment remain unclarified. We assessed electrophysiology of central motor and sensory conduction in patients with SS before and after iron chelator administration. In this controlled trial, we evaluated 12 patients with SS (mean follow-up: 33.6 months [range: 18.9-36.1]), who underwent dural repair, and 9 with multiple sclerosis as controls. Transcranial magnetic stimulation (TMS) and somatosensory evoked potentials (SEPs) were used to assess motor and sensory conduction, respectively. Eight patients with SS received oral iron chelator deferiprone (1,500 mg/day), whereas four underwent surgical repair without iron chelation. Primary outcomes were changes in central motor conduction time (CMCT), motor evoked potential (MEP) amplitude, and central sensory conduction time (CCT). At baseline, 92 and 100% of patients with SS exhibited prolonged CMCT and CCT, respectively. Prolonged conduction in patients with SS was less severe than in those with multiple sclerosis (MS). Whereas MEP amplitude and the amplitude ratio of MEP to CMAP were relatively preserved in patients with SS. After 36-month iron chelator administration, statistically significant improvement of central motor conduction time was gained in patients with SS. TMS and SEP are sensitive tools for evaluating central conduction impairment in SS. The conduction abnormality, likely caused by a different pathomechanism from that in MS, is partially recoverable by iron chelator administration. CMCT improvement demonstrates partial reversibility of impaired motor pathway and supports the therapeutic potential of iron chelator therapy in SS.

#3

Lifelong trajectories of modifiable vascular risk factors and relation to cerebral small vessel disease in the Framingham Heart Study.

medRxiv : the preprint server for health sciences2026 Jan 19

Cerebral small vessel disease (CSVD) is strongly linked to stroke and dementia risk, frequently predating clinical events for years to decades. Preventive efforts focus on treatment of modifiable vascular risk factors (VRF), but the complex changes in VRF over long periods in relation to CSVD burden remain unclear. Thus, we aimed to characterize life-long VRF trajectories in community-dwelling individuals and relate them to CSVD burden. Framingham Heart Study participants from the Original and Offspring cohorts with six or more repeated VRF assessments over their lifetime were eligible for the present study. Among those who underwent MRI imaging, CSVD burden was quantified by assigning one point each for cerebral microbleeds, covert infarcts, extensive white matter hyperintensities, cortical superficial siderosis, and high perivascular space burden (range: 0-5). VRF trajectories and trajectory-based clusters were then examined in relation to CSVD burden using functional regression analysis. Of 7,961 participants with longitudinal VRF measurements (mean baseline age 39.8 ± 10 years, 45% men), 1,625 underwent MRI imaging after exclusions for other neurological conditions. In models that used individual VRF trajectories as covariates, systolic and diastolic blood pressure, pulse pressure, cigarettes per day and triglycerides were significantly associated with CSVD burden later in life. In a complementary analysis, we clustered VRF trajectories and then used each participant's cluster assignment (rather than the raw trajectories) as the covariate; cluster assignments diverged early in life for systolic blood pressure and pulse pressure and were significantly associated with CSVD burden. Individuals following high risk lifetime patterns of vascular risk factors have higher CSVD burden later in life. These results highlight the importance of primordial and primary prevention with sustained risk factor management across the lifespan to mitigate CSVD burden, a strong determinant of stroke and dementia risk.

#4

Cerebral microbleed patterns and the risk of incident dementia in elderly adults: The ARIC study.

PloS one2026

The association between cerebral microbleeds (CMBs), an indicator of microvascular damage on MRI, and incident dementia (ID) remains inconclusive. We aim to investigate the interplay of CMB presence, number, and location with ID in a large community-based elderly cohort. The study included 1532 dementia-free participants (aged ≥ 65 years) derived from baseline examinations (2012-2013) of the Atherosclerosis Risk in Communities Neurocognitive Study (ARIC-NCS). The ID cases were diagnosed at visits 6 (2016-2017), 7 (2018-2019), and 8 (2019-2020). Cox proportional-hazards models were deployed to assess the association of CMB presence, number, and location with ID. Over a nine-year follow-up (median = 8.1 years), 390 participants developed ID. Microbleed (MB) presence was related to an increased ID risk (HR = 1.24, 95% CI = 1.02-1.51). A stronger association was observed in those with mixed (lobar+subcortical) MBs (HR = 1.60, 95% CI = 1.10-2.30). Compared to participants without MBs, those with ≥ 2 CMBs in any MBs, any lobar MBs, and any subcortical MBs were associated with 57%, 174%, and 74% higher ID risks. Furthermore, those with a pattern of only lobar MBs or superficial siderosis had the highest ID risk (HR = 1.80, 95% CI = 1.26-2.65). In this large community-based elderly cohort, we identified that the presence of MBs or a high MB count (i.e., ≥ 2), with some specificity for location, was independently related to an increased ID risk over a nine-year follow-up.

#5

Prevalence of vestibulo-ocular reflex dysfunction in people with neurological disorders: a systematic review and meta-analysis.

Journal of neurology2026 Jan 21

Neurological disorders, a leading cause of global disability, often cause debilitating dizziness and imbalance. While subjective symptoms are well-documented, the actual prevalence of vestibulo-ocular reflex (VOR) dysfunction in patients with central nervous system (CNS) damage remains unclear due to inconsistent primary studies. This research aims to determine the prevalence of VOR gain dysfunction, as measured by the video Head Impulse Test (vHIT), across neurological disorders. Our systematic review searched MEDLINE, CENTRAL, CINAHL, Scopus, ClinicalTrials.gov, and the WHO ICTRP for original articles from 2009 to September 2025. The JBI Checklist for prevalence studies was used to assess the methodological quality, and descriptive analyses were performed, followed by a meta-analysis of proportions using a random-intercept logistic regression model. We included 48 studies, of which three reported on the same or overlapping samples. Thus, 45 unique studies (1604 participants, 792 females, mean age 56) were described. A meta-analysis of 33 studies (1129 participants) found an overall prevalence of vestibular dysfunction of 48% (95% CI 31-67%). Given the high heterogeneity, we performed subgroup analyses by condition. We found a pooled prevalence of 98% for CANVAS, 73% for ataxia, 44% for Parkinson's disease, 59% for multiple sclerosis, 15% for traumatic brain injury, 5% for multiple system atrophy, and 77% for superficial siderosis. Isolated semicircular canal dysfunctions, as documented using vHIT, are prevalent in neurological disorders. Future research must elucidate their etiology and diagnostic potential, utilizing comprehensive vestibular assessments. Eventually, these findings should be translated into improved, evidence-based rehabilitation strategies. CRD42024575542.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC560 artigos no totalmostrando 197

2026

Interim analysis of all-case post-marketing surveillance study in Japan: lecanemab in patients with early Alzheimer's disease.

The journal of prevention of Alzheimer's disease
2026

Clinical retrospective analysis of 14 cases of superficial siderosis of the central nervous system.

BMC neurology
2026

Linking juxtacortical perivascular spaces to cerebral amyloid angiopathy: A study based on 5.0 T MRI.

Journal of Alzheimer's disease : JAD
2026

Unique presentation of superficial siderosis of the central nervous system following pituitary tumor surgery: a case report and literature review.

Frontiers in neuroimaging
2026

[Amyloid β-related angiitis (ABRA) with dural contrast enhancement].

Rinsho shinkeigaku = Clinical neurology
2026

Two autopsy cases of superficial siderosis: ultrastructural similarity across traumatic and non-traumatic contexts and forensic uncertainties.

Legal medicine (Tokyo, Japan)
2026

Quantitative evaluation of iron chelator effects on central motor and sensory tracts in superficial siderosis.

Frontiers in neurology
2026

Perivascular Spaces Are Associated With CSF Aβ in Cerebral Amyloid Angiopathy But Not in Deep Perforator Arteriopathy.

Stroke
2026

Prevalence of covert cerebrovascular changes in schizophrenia: A systematic review and meta-analysis.

Schizophrenia research
2026

Associations Between Cerebral Microbleed Patterns and Incident Dementia: The ARIC-Neurocognitive Study.

Stroke
2026

Topography and cognitive impact of brain iron deposition in cerebral amyloid angiopathy: Evidence from 7T quantitative susceptibility mapping.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2026

The association of Apolipoprotein E ε4 with symptomatic intracerebral hemorrhage, MRI markers and cognition in Dutch-type hereditary cerebral amyloid angiopathy.

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association
2026

Low-intensity rim on T2-weighted brainstem imaging: a universally observed structure exhibiting a negative magnetic susceptibility effect.

Japanese journal of radiology
2026

Probable cerebral amyloid angiopathy - related inflammation in a 32-year-old woman with down syndrome.

Neurogenetics
2026

Lifelong trajectories of modifiable vascular risk factors and relation to cerebral small vessel disease in the Framingham Heart Study.

medRxiv : the preprint server for health sciences
2026

Contrasting patterns of leptomeningeal and parenchymal gadolinium extravasation in cerebral amyloid angiopathy: An MRI-based evaluation.

International journal of stroke : official journal of the International Stroke Society
2026

[Infratentorial superficial siderosis : a diagnostic challenge from the general practice to the hospital].

Revue medicale suisse
2025

Aggressive biological behavior of benign central nervous system neoplasms: The case of hemorrhagic subependymomas - An illustrated review.

Surgical neurology international
2026

Cerebral Amyloid Angiopathy Imaging Markers, Radiological Boston Criteria, and Thrombolysis Outcomes in Ischemic Stroke.

Stroke
2026

Cerebral microbleed patterns and the risk of incident dementia in elderly adults: The ARIC study.

PloS one
2026

Prevalence of vestibulo-ocular reflex dysfunction in people with neurological disorders: a systematic review and meta-analysis.

Journal of neurology
2026

Imaging and fluid biomarkers for prognostic stratification in cerebral amyloid angiopathy.

Journal of neurology
2026

A regional framework for the detection and management of ARIA with anti-amyloid therapies in early Alzheimer's disease in Asia.

The journal of prevention of Alzheimer's disease
2026

Meningovascular Inflammation in Cerebral Amyloid Angiopathy-Related Cortical Superficial Siderosis.

Annals of clinical and translational neurology
2025

The Storm Behind the Silence: Detecting Cerebral Amyloid Angiopathy-Related Inflammation in a Patient with Dementia.

Journal of Brown hospital medicine
2025

Progressive Bilateral Sensorineural Hearing Loss and Ataxia Caused by Superficial Siderosis Secondary to Chronic Spontaneous Intracranial Hypotension.

Cureus
2026

Severe symptomatic amyloid-related imaging abnormalities in Alzheimer's disease: Two case reports and systematic review of reported cases.

Journal of Alzheimer's disease : JAD
2025

18F-MK-6240 uptake in cortical tau and hemorrhagic lesions in a case of Alzheimer's disease with possible crossed aphasia.

eNeurologicalSci
2025

Biopsy-Confirmed Cerebral Amyloid Angiopathy-Related Inflammation With Absence of Cerebral Microbleeds on T2* and Detectable Only by Susceptibility-Weighted Imaging.

Cureus
2025

Pathological findings from surgical specimens and long-term outcome in cerebral amyloid angiopathy patients with intracerebral hemorrhage.

Scientific reports
2025

Cognitive Age Delta as a Marker of Healthy and Pathological Cognitive Aging: The Role of Lifestyle, Cognitive Reserve, and Vascular Risk.

Journal of clinical medicine
2025

Iatrogenic cerebral amyloid angiopathy 30 years after childhood cadaveric dura mater transplantation: a case report.

BMC neurology
2025

Duropathies: A Narrative Overview of a Neglected Concept-Part One: Anatomical, Embryological, and Pathophysiological Elements.

NeuroSci
2025

Profiles of blood-brain barrier and neurodegeneration markers in cerebrospinal fluid of patients with cerebral amyloid angiopathy.

Alzheimer's &amp; dementia (Amsterdam, Netherlands)
2026

Electroconvulsive Therapy for Refractory Anxiety and Depression in Superficial Siderosis: A Case Report.

Clinical neuropharmacology
2025

White Matter Free Water and PSMD as Neuroimaging Biomarkers of Cerebral Amyloid Angiopathy Severity.

Neurology
2025

Iatrogenic cerebral amyloid angiopathy with inflammatory pathogenesis: A case report and literature review.

Surgical neurology international
2025

Neuroimaging Markers Associated With Recurrent Stroke in Intracerebral Hemorrhage and Atrial Fibrillation: Secondary Analysis of PRESTIGE-AF.

Neurology
2025

Higher Burden of Cerebral Small Vessel Disease Is Associated With Risk of Incident Stroke in Community-Dwelling Individuals.

Journal of the American Heart Association
2025

[Ataxia, deafness and diplopia].

La Revue de medecine interne
2025

To select or not to select: evaluating the effectiveness of SeLECT score in predicting the risk of post-stroke seizures: a systematic review.

Epilepsy &amp; behavior : E&amp;B
2025

In Vivo Imaging of Blood-Brain Barrier Leakage Using a Contrast Agent in Patients With Cerebral Amyloid Angiopathy: An Exploratory Study.

Neurology
2025

Cerebral amyloid angiopathy-related inflammation superimposed on alpha-synucleinopathies: a case report and literature review.

Journal of neuroimmunology
2025

Determinants of cerebral amyloid angiopathy progression: a multicenter retrospective study.

Journal of neurology
2025

Microvascular brain damage in middle-aged women with a history of migraine with aura and/or ischemic stroke.

International journal of stroke : official journal of the International Stroke Society
2025

Cerebrospinal Fluid Biomarkers Profiling in Cerebral Amyloid Angiopathy and Relationship With Disease Phenotypes.

Journal of the American Heart Association
2025

Superficial siderosis in Marfan syndrome.

Practical neurology
2025

Safety and effectiveness of lecanemab in Chinese patients with early Alzheimer's disease: Evidence from a multidimensional real-world study.

Chinese medical journal
2025

Amyloid-related Imaging Abnormalities (ARIA) in the Context of Alzheimer's Disease and Amyloid-targeting Therapies: An Introduction for Advanced Practice Providers.

Drugs &amp; aging
2025

Iatrogenic superficial siderosis with the Pseudomeningocele after spinal surgery: a report of four cases and literature review.

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
2025

Multimarker Cerebral Small Vessel Disease Score and Risk of Incident Dementia in the Framingham Heart Study.

Neurology
2026

The role of brain MR and FDG-PET in the diagnosis of neurodegenerative disease.

European radiology
2025

When Blood Leaves a Trace: Diagnosing Superficial Siderosis in a Geriatric Patient.

Cureus
2025

SATURN MRI: study protocol for the statin use in intracerebral hemorrhage patients MRI ancillary study.

Trials
2025

Prevalent Cardiovascular Disease and Atrial Fibrillation in Relation to Cerebral Small Vessel Disease Burden.

Brain sciences
2025

Coexisting Subdural Hematoma in Cerebral Amyloid Angiopathy: A Case Series.

Neurology international
2026

Multi-marker cerebral small vessel disease score and risk of incident depression: The Framingham Heart Study.

Journal of affective disorders
2025

Prevalence and risk factors of cerebral microhemorrhages and superficial siderosis in cognitively unimpaired older adults: analysis from the CHARIOT-PRO SubStudy.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2025

Diabetic Ketosis Complicated with Superficial Siderosis.

Neurology India
2025

Intraventricular cavernous hemangioma hemorrhage leads to Superficial Siderosis of the Central Nervous System.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2025

Cerebral Amyloid Angiopathy-Related Inflammation: Clinical Characteristics and Treatment Experience.

Clinical interventions in aging
2025

Spinal Myxopapillary Ependymoma Presenting With Isolated Intracranial Hypertension: A Case Report.

Cureus
2026

MRI Findings of Intracranial Hemorrhages in ARCADIA-MRI: An Ancillary Study to the ARCADIA Trial.

AJNR. American journal of neuroradiology
2025

Accelerated MRI Sequences for Intracranial Hemorrhage Screening.

Investigative radiology
2025

Central nervous system superficial siderosis: A case report and literature review.

Medicine
2025

Cerebral Amyloid Angiopathy With Progressive Cortical Superficial Siderosis and Convexity Subarachnoid Hemorrhage in an Apolipoprotein E (APOE) ε2/ε2 Homozygous Patient: A Case Report and Literature Review.

Cureus
2025

Audiological Progression and Treatment Outcomes in Superficial Siderosis: A 10-Year Retrospective Review of 46 Patients.

Otology &amp; neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
2025

Clinical Management of Cerebral Amyloid Angiopathy.

Journal of clinical medicine
2025

Outcomes of CT-Guided Targeted Epidural Patching for Lateral Dural Tears in Spontaneous Intracranial Hypotension: A Multicenter Retrospective Cohort Study.

AJNR. American journal of neuroradiology
2025

Monitoring of amyloid related imaging abnormalities: SWI vs T2*-GRE.

The journal of prevention of Alzheimer's disease
2025

Prediction Model to Optimize Long-Term Antithrombotic Therapy Using Covert Vascular Brain Injury and Clinical Features.

Stroke
2025

"Real-world" eligibility for anti-amyloid treatment in a tertiary memory clinic setting.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2025

MRI protocols and sequences for amyloid-related imaging abnormalities monitoring in Alzheimer's disease patients treated with monoclonal antibodies.

Current opinion in neurology
2026

Vascular β-amyloid potentially colocalises with phosphorylated tau in cerebral amyloid angiopathy.

Stroke and vascular neurology
2025

Clinical Features and Diagnosis of Spontaneous Intracranial Hypotension.

Continuum (Minneapolis, Minn.)
2025

Cerebral Amyloid Angiopathy: A Case Report.

Cureus
2025

Iatrogenic Cerebral Amyloid Angiopathy After Childhood Brain Surgery: Novel Findings of MRI and CT.

Neurology international
2025

Cochlear Implantation: A Treatment Approach of Hearing Loss in Superficial Siderosis.

The journal of international advanced otology
2025

Lecanemab Treatment in a Specialty Memory Clinic.

JAMA neurology
2025

Cerebral Amyloid Angiopathy and Downstream Alzheimer Disease Plasma Biomarkers.

JAMA network open
2025

CSF-venous fistulae-An underrecognized cause of superficial siderosis.

Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences
2025

Added Value of Cerebral Small Vascular Disease Burden Based On MRI for Prediction Poor Outcome of Intracerebral Hemorrhage.

Clinical neuroradiology
2025

Re-evaluation of the efficacy and safety of anti-Aβ monoclonal antibodies (lecanemab/donanemab) in the treatment of early Alzheimer's disease.

Frontiers in pharmacology
2025

New-Onset Progressive Ataxia in a 66-Year-Old Woman with Longstanding Parkinson's Disease: A Case Report.

Case reports in neurology
2025

One-Year Radiologic Progression in Sporadic and Hereditary Cerebral Amyloid Angiopathy.

Neurology
2025

Seizures in Cerebral Amyloid Angiopathy: A Systematic Review and Meta-Analysis.

Neurology. Clinical practice
2025

Donanemab: Appropriate use recommendations.

The journal of prevention of Alzheimer's disease
2025

Cerebral Amyloid Angiopathy: Clinical Presentation, Sequelae and Neuroimaging Features-An Update.

Biomedicines
2025

Safety and efficacy of long-term gantenerumab treatment in dominantly inherited Alzheimer's disease: an open-label extension of the phase 2/3 multicentre, randomised, double-blind, placebo-controlled platform DIAN-TU trial.

The Lancet. Neurology
2025

Maximizing the benefit and managing the risk of anti-amyloid monoclonal antibody therapy for Alzheimer's disease: Strategies and research directions.

Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics
2025

Incidence of Amyloid-Related Imaging Abnormalities in Phase III Clinical Trials of Anti-Amyloid-β Immunotherapy: An Updated Meta-Analysis.

Neurology
2025

Clinical and Neuroradiological Manifestations of Cerebral Amyloid Angiopathy: A Closer Look into the Natural History of a Frequent Disease.

Journal of clinical medicine
2025

Spinal myxopapillary ependymoma presenting with intracranial hypertension.

Practical neurology
2025

Amyloid-Related Imaging Abnormalities With Donanemab in Early Symptomatic Alzheimer Disease: Secondary Analysis of the TRAILBLAZER-ALZ and ALZ 2 Randomized Clinical Trials.

JAMA neurology
2025

Prevalence of infratentorial superficial siderosis in a large general population sample from the UK Biobank.

Journal of neurology
2025

Long-Term Cognitive Decline After Subarachnoid Hemorrhage: Pathophysiology, Management, and Future Directions.

Stroke
2025

Cerebrospinal Fluid Beta-Amyloid Concentration and Clinical and Radiographic Manifestations of Cerebral Amyloid Angiopathy.

Journal of the American Heart Association
2025

Explainable models for predicting long-term outcomes in patients with spontaneous intracerebral haemorrhage: a retrospective cohort study.

Stroke and vascular neurology
2025

A Case of Cerebral Amyloid Angiopathy With Recurrent Hemorrhagic and Ischemic Strokes Under Direct Oral Anticoagulant Therapy for Atrial Fibrillation.

Cureus
2025

[Clinical characteristics and prognosis of type 1 infratentorial superficial siderosis].

Zhonghua yi xue za zhi
2025

Teaching NeuroImage: Superficial Siderosis in Marfan Syndrome.

Neurology
2025

Cerebral Amyloid Angiopathy in Alzheimer Disease: A Comparison Between Different Versions of the Boston Criteria.

Neurology
2025

Clinical diagnosis of cerebral amyloid angiopathy related hemorrhage in China: Simplified Edinburgh criteria and Boston criteria version 2.0.

European stroke journal
2025

Amyloid-related imaging abnormalities: manifestations, metrics and mechanisms.

Nature reviews. Neurology
2025

Infratentorial superficial siderosis due to a spinal dural tear.

Practical neurology
2024

Incidental asymptomatic spinal cerebrospinal fluid leaks: single-center experience, and a presentation of seven cases.

Journal of neurointerventional surgery
2026

Insights into the transcriptomic heterogeneity of brain endothelial cells in normal aging and Alzheimer's disease.

Neural regeneration research
2024

Cognitive Impairment in Cerebral Amyloid Angiopathy: A Single-Center Prospective Cohort Study.

Journal of clinical medicine
2024

Higher burden of cerebral small vessel disease is associated with risk of incident stroke in community dwelling individuals.

medRxiv : the preprint server for health sciences
2025

Amyloid-Related Imaging Abnormalities (ARIA) in Clinical Trials of Gantenerumab in Early Alzheimer Disease.

JAMA neurology
2024

MRI-Based Prediction of Macrovascular Causes of Intracerebral Hemorrhage: The MACRO Score.

Neurology
2024

Spinal subarachnoid hematoma involving a thoracic ventral dural defect: illustrative case.

Journal of neurosurgery. Case lessons
2024

Superficial Siderosis: A Case Report of Underdiagnosed Disorder.

Cureus
2024

Acute to Chronic Variants in the Imaging Spectrum of Superficial Siderosis: Case Series and Literature Review.

Cureus
2024

YAP1 fusion-positive ependymoma presenting in an adult with a pigmented phenotype and association with superficial siderosis.

Free neuropathology
2025

Ventral Thoracic Cerebrospinal Fluid Leak Repair in Spontaneous Intracranial Hypotension Complicated by Superficial Siderosis: 2-Dimensional Operative Video.

Operative neurosurgery (Hagerstown, Md.)
2024

[A case of young onset cerebral amyloid angiopathy associated with dural grafting].

Rinsho shinkeigaku = Clinical neurology
2024

From Spinal Ependymoma to Superficial Siderosis: A Bottom to Top Cause of Progressive Neurological Deterioration.

The Neurohospitalist
2024

Duropathy as a rare motor neuron disease mimic: from bibrachial amyotrophy to infratentorial superficial siderosis.

BMC neurology
2024

Brain MRI in patients with V30M hereditary transthyretin amyloidosis.

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
2024

Amyloid-Related Imaging Abnormalities in the Era of Anti-Amyloid Beta Monoclonal Antibodies for Alzheimer's Disease: Recent Updates on Clinical and Imaging Features and MRI Monitoring.

Korean journal of radiology
2024

Clinical and neuroimaging precursors in cerebral amyloid angiopathy: impact of the Boston criteria version 2.0.

European journal of neurology
2024

A Case of Idiopathic Intracranial Hypertension Complicated with both Infratentorial and Supratentorial Cortical Superficial Siderosis: Novel Imaging Findings on Intravoxel Incoherent Motion Magnetic Resonance Imaging Offering Clues to Pathophysiology.

Neurology international
2024

Application of artificial intelligence-based magnetic resonance imaging in diagnosis of cerebral small vessel disease.

CNS neuroscience &amp; therapeutics
2024

Association of enlarged perivascular spaces with cognitive function in dementia-free older adults: A population-based study.

Alzheimer's &amp; dementia (Amsterdam, Netherlands)
2024

Tumefactive cerebral amyloid angiopathy - related inflammation.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2024

The hidden link between late-onset seizures and cerebral amyloid angiopathy: A case-control study.

Epilepsia open
2025

Spontaneous intracranial hypotension - a spinal disease.

RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin
2024

Occult Amyloid-β-Related Angiitis: Neuroimaging Findings at 1.5T, 3T, and 7T MRI.

AJNR. American journal of neuroradiology
2024

A Case of Superficial Siderosis with Elevated Anti-Ro/SSA Antibody.

NMC case report journal
2024

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

Neurology
2024

Superficial Siderosis of the Central Nervous System: A Report of Two Cases With Spinal Pathology and a Review of the Literature.

Cureus
2024

Non-hemorrhagic imaging markers of cerebral amyloid angiopathy in memory clinic patients.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2024

Quantitative electroencephalography in cerebral amyloid angiopathy.

Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology
2024

Clinical characteristics of cerebral amyloid angiopathy and risk factors of cerebral amyloid angiopathy related intracerebral hemorrhage.

Journal of neurology
2024

Central Nervous System Siderosis Associated with Multiple Cerebral Aneurysms: Literature Review and Description of an Additional Case.

World neurosurgery
2024

Pathophysiology, Diagnosis, Prognosis, and Prevention of Poststroke Epilepsy: Clinical and Research Implications.

Neurology
2025

Superficial Siderosis Causing Refractory Papilledema Following Brain Tumor Resection.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2024

Spinal intradural pseudocyst formation in central nervous system superficial siderosis.

Brain pathology (Zurich, Switzerland)
2024

Detection of Dural Defect Localization Using 4-Dimensional Dynamic Computed Tomography Myelography for Patients with Superficial Siderosis.

World neurosurgery
2024

Sex differences in histopathological markers of cerebral amyloid angiopathy and related hemorrhage.

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

Hemorrhagic Transformation in Noncardioembolic Acute Ischemic Stroke: MRI Analysis From PACIFIC-STROKE.

Stroke
2024

Cerebral tau pathology in cerebral amyloid angiopathy.

Brain communications
2024

Progressive superficial siderosis despite complete remission of intracranial hypotension symptoms following epidural patching: Case report.

Headache
2024

Spontaneous Intracranial Hypotension: Clinical Presentation, Diagnosis, and Treatment Strategies.

Neurologic clinics
2024

Superficial Siderosis After Traumatic Brain Injury: A Case Report.

Cureus
2024

Superficial small cerebellar infarcts in cerebral amyloid angiopathy on 3 T MRI: A preliminary study.

Journal of the neurological sciences
2024

Lumbar Subarachnoid-Peritoneal Shunting Deteriorates Superficial Siderosis Associated with a Dural Defect.

Cureus
2024

Corrigendum: Infratentorial superficial siderosis: report of six cases and review of the literature.

Frontiers in neuroscience
2024

[Cerebral amyloid angiopathy].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
2024

Cardiovascular Management in Asymptomatic (Silent) Cerebral Microbleeds and Suspected Cerebral Amyloid Angiopathy.

Stroke
2024

Temporal Ordering of Biomarkers in Dutch-Type Hereditary Cerebral Amyloid Angiopathy.

Stroke
2024

Cerebral Superficial Siderosis.

Neurology India
2024

Infratentorial superficial siderosis: report of six cases and review of the literature.

Frontiers in neuroscience
2025

Advances in Cerebral Small Vessel Disease: Sandra E. Black Lecture to the Canadian Neurological Sciences Federation.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2024

The Association Between Positive Amyloid-PET and Cognitive Decline Is Not Always Supportive of Alzheimer's Disease: Suggestions from a Case Report.

Journal of Alzheimer's disease reports
2024

Spinal Anterior Dural Dissection: Moving From Differential to Unifying Diagnosis.

Neurosurgery
2024

Artificial Intelligence Assistive Software Tool for Automated Detection and Quantification of Amyloid-Related Imaging Abnormalities.

JAMA network open
2023

Research landscape and trends of cerebral amyloid angiopathy: a 25-year scientometric analysis.

Frontiers in neurology
2024

Deep learning applications in vascular dementia using neuroimaging.

Current opinion in psychiatry
2024

Post-transfusion severe headache in a patient with thalassemia with superficial siderosis of the central nervous system: a case report and literature review.

BMC neurology
2024

Genome-Wide Association Studies of ARIA From the Aducanumab Phase 3 ENGAGE and EMERGE Studies.

Neurology
2024

Superficial siderosis and the dura.

European journal of neurology
2023

[Cerebral Small Vessel Disease: Advances in Understanding its Pathophysiology].

Fortschritte der Neurologie-Psychiatrie
2024

Left atrial appendage occlusion in patients with spontaneous intracerebral hemorrhage: An observational study.

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association
2024

SWI versus GRE-T2*: Assessing cortical superficial siderosis in advanced cerebral amyloid angiopathy.

Revue neurologique
2023

Risk factors for recurrent cerebral amyloid angiopathy-related intracerebral hemorrhage.

Frontiers in neurology
2023

The FDA-approved anti-amyloid-β monoclonal antibodies for the treatment of Alzheimer's disease: a systematic review and meta-analysis of randomized controlled trials.

European journal of medical research
2024

Surgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis.

European journal of neurology
2024

Combined effect of cortical superficial siderosis and cerebral microbleed on short-term and long-term outcomes after intracerebral haemorrhage.

Stroke and vascular neurology
2022

[Superficial siderosis of the central nervous system. Report of one case].

Revista medica de Chile
2023

Apraxia Associated With Superficial Siderosis in Cerebral Amyloid Angiopathy.

Journal of clinical neurology (Seoul, Korea)
2024

Superficial siderosis of the central nervous system caused by myxopapillary ependymoma of cauda equine: a case report.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2023

Etiology of Primary Cerebellar Intracerebral Hemorrhage Based on Topographic Localization.

Stroke
2023

Detection rate of brain MR and MR angiography for neuroimaging abnormality in patients with newly diagnosed left-sided infective endocarditis.

Scientific reports
2024

[Analysis of the prevalence of anticoagulant therapy in patients with cognitive disorders and cerebral amyloid angiopathy (CAA)].

Der Nervenarzt
2023

A superficial siderosis-related transient focal neurological episode in a patient with Marfan syndrome.

Chronic diseases and translational medicine
2023

A case report of superficial siderosis of the central nervous system and literature review.

The Journal of international medical research
2023

Superficial Cerebellar Siderosis and Spontaneous Intracranial Hypotension Secondary to Dural Tear.

The Neurohospitalist
2023

Clinical Characteristics, Neuroimaging Markers, and Outcomes in Patients with Cerebral Amyloid Angiopathy: A Prospective Cohort Study.

Journal of clinical medicine
2023

Cerebral Microbleed Patterns and Cortical Amyloid-β: The ARIC-PET Study.

Stroke
2023

Cortical superficial siderosis is associated with reactive astrogliosis in cerebral amyloid angiopathy.

Journal of neuroinflammation
2023

Impact of previous statin use on first intracerebral hemorrhage in cerebral amyloid angiopathy.

Revue neurologique
2023

Postoperative vasospasm and cerebral infarction in a patient with large pituitary adenoma and cerebral superficial siderosis.

Surgical neurology international
2023

[Classical cortical superficial siderosis presenting as extensive higher brain dysfunction with hypoperfusion in the frontoparietal lobe on the 123I-IMP-SPECT: a case report].

Rinsho shinkeigaku = Clinical neurology
2023

Cognitive Versus Hemorrhagic Onset in Cerebral Amyloid Angiopathy: Neuroimaging Features.

Current Alzheimer research
2023

Age-related macular degeneration is associated with probable cerebral amyloid angiopathy: A case-control study.

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association
2023

Preceding symptoms and temporal development of cortical superficial siderosis in cerebral amyloid angiopathy: a case report.

BMC neurology
2023

Association of the Presence and Pattern of MRI Markers of Cerebral Small Vessel Disease With Recurrent Intracerebral Hemorrhage.

Neurology
2023

Mechanistic Implications of Cortical Superficial Siderosis in Patients With Mixed Location Intracerebral Hemorrhage and Cerebral Microbleeds.

Neurology
2023

Quantitative measurement of cortical superficial siderosis in cerebral amyloid angiopathy.

NeuroImage. Clinical
2023

Incidence and risk factors associated with seizures in cerebral amyloid angiopathy.

European journal of neurology
2023

Superficial siderosis: comparison of two cases indicates two distinct diagnostic entities. Illustrative cases.

Journal of neurosurgery. Case lessons
2023

Case report of iatrogenic cerebral amyloid angiopathy after exposure to Lyodura: an Australian perspective.

Frontiers in neuroscience
2023

Cortical superficial siderosis, hematoma volume, and outcomes after intracerebral hemorrhage: a mediation analysis.

Frontiers in neurology
2023

Amyloid and tau PET in cerebral amyloid angiopathy-related inflammation two case reports and literature review.

Frontiers in neurology
2023

Phenotype and imaging features associated with APP duplications.

Alzheimer's research &amp; therapy
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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. [Amyloid &#x3b2;-related angiitis (ABRA) with dural contrast enhancement].
    Rinsho shinkeigaku = Clinical neurology· 2026· PMID 41833369mais citado
  2. Quantitative evaluation of iron chelator effects on central motor and sensory tracts in superficial siderosis.
    Frontiers in neurology· 2026· PMID 41789166mais citado
  3. Lifelong trajectories of modifiable vascular risk factors and relation to cerebral small vessel disease in the Framingham Heart Study.
    medRxiv : the preprint server for health sciences· 2026· PMID 41646706mais citado
  4. Cerebral microbleed patterns and the risk of incident dementia in elderly adults: The ARIC study.
    PloS one· 2026· PMID 41564004mais citado
  5. Prevalence of vestibulo-ocular reflex dysfunction in people with neurological disorders: a systematic review and meta-analysis.
    Journal of neurology· 2026· PMID 41563462mais citado
  6. Giant cauda equina neuroendocrine tumor with superficial siderosis: illustrative case.
    J Neurosurg Case Lessons· 2026· PMID 41974061recente
  7. Posterior confluent white matter hyperintensities and intracerebral hemorrhage recurrence risk in patients with cerebral amyloid angiopathy.
    Front Neurol· 2026· PMID 41908271recente
  8. Duropathies as Unifying Concept-Part Two: A Narrative Overview of Clinical and Neuroradiological Features.
    Neurol Int· 2026· PMID 41893062recente
  9. Abducens nerve palsy as a false localising sign in spontaneous intracranial hypotension.
    BMJ Case Rep· 2026· PMID 41881482recente
  10. Interim analysis of all-case post-marketing surveillance study in Japan: lecanemab in patients with early Alzheimer's disease.
    J Prev Alzheimers Dis· 2026· PMID 41849958recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:247245(Orphanet)
  2. MONDO:0016594(MONDO)
  3. GARD:9484(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q7643329(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

Siderose superficial
Compêndio · Raras BR

Siderose superficial

ORPHA:247245 · MONDO:0016594
Prevalência
Unknown
Casos
300 casos conhecidos
Herança
Not applicable
CID-10
I69.0 · Seqüelas de hemorragia subaracnoídea
Ensaios
2 ativos
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C2938918
EuropePMC
Wikidata
Papers 10a
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