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.
Introdução
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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.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 28 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 49 características clínicas mais associadas, ordenadas por frequência.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
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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 — Siderose superficial
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Publicações mais relevantes
[Amyloid β-related angiitis (ABRA) with dural contrast enhancement].
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.
Quantitative evaluation of iron chelator effects on central motor and sensory tracts in superficial siderosis.
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.
Lifelong trajectories of modifiable vascular risk factors and relation to cerebral small vessel disease in the Framingham Heart Study.
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.
Cerebral microbleed patterns and the risk of incident dementia in elderly adults: The ARIC study.
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.
Prevalence of vestibulo-ocular reflex dysfunction in people with neurological disorders: a systematic review and meta-analysis.
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
Giant cauda equina neuroendocrine tumor with superficial siderosis: illustrative case.
Posterior confluent white matter hyperintensities and intracerebral hemorrhage recurrence risk in patients with cerebral amyloid angiopathy.
Duropathies as Unifying Concept-Part Two: A Narrative Overview of Clinical and Neuroradiological Features.
Abducens nerve palsy as a false localising sign in spontaneous intracranial hypotension.
Interim analysis of all-case post-marketing surveillance study in Japan: lecanemab in patients with early Alzheimer's disease.
📚 EuropePMC560 artigos no totalmostrando 197
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 diseaseClinical retrospective analysis of 14 cases of superficial siderosis of the central nervous system.
BMC neurologyLinking juxtacortical perivascular spaces to cerebral amyloid angiopathy: A study based on 5.0 T MRI.
Journal of Alzheimer's disease : JADUnique presentation of superficial siderosis of the central nervous system following pituitary tumor surgery: a case report and literature review.
Frontiers in neuroimaging[Amyloid β-related angiitis (ABRA) with dural contrast enhancement].
Rinsho shinkeigaku = Clinical neurologyTwo autopsy cases of superficial siderosis: ultrastructural similarity across traumatic and non-traumatic contexts and forensic uncertainties.
Legal medicine (Tokyo, Japan)Quantitative evaluation of iron chelator effects on central motor and sensory tracts in superficial siderosis.
Frontiers in neurologyPerivascular Spaces Are Associated With CSF Aβ in Cerebral Amyloid Angiopathy But Not in Deep Perforator Arteriopathy.
StrokePrevalence of covert cerebrovascular changes in schizophrenia: A systematic review and meta-analysis.
Schizophrenia researchAssociations Between Cerebral Microbleed Patterns and Incident Dementia: The ARIC-Neurocognitive Study.
StrokeTopography and cognitive impact of brain iron deposition in cerebral amyloid angiopathy: Evidence from 7T quantitative susceptibility mapping.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationThe 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 AssociationLow-intensity rim on T2-weighted brainstem imaging: a universally observed structure exhibiting a negative magnetic susceptibility effect.
Japanese journal of radiologyProbable cerebral amyloid angiopathy - related inflammation in a 32-year-old woman with down syndrome.
NeurogeneticsLifelong trajectories of modifiable vascular risk factors and relation to cerebral small vessel disease in the Framingham Heart Study.
medRxiv : the preprint server for health sciencesContrasting 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[Infratentorial superficial siderosis : a diagnostic challenge from the general practice to the hospital].
Revue medicale suisseAggressive biological behavior of benign central nervous system neoplasms: The case of hemorrhagic subependymomas - An illustrated review.
Surgical neurology internationalCerebral Amyloid Angiopathy Imaging Markers, Radiological Boston Criteria, and Thrombolysis Outcomes in Ischemic Stroke.
StrokeCerebral microbleed patterns and the risk of incident dementia in elderly adults: The ARIC study.
PloS onePrevalence of vestibulo-ocular reflex dysfunction in people with neurological disorders: a systematic review and meta-analysis.
Journal of neurologyImaging and fluid biomarkers for prognostic stratification in cerebral amyloid angiopathy.
Journal of neurologyA 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 diseaseMeningovascular Inflammation in Cerebral Amyloid Angiopathy-Related Cortical Superficial Siderosis.
Annals of clinical and translational neurologyThe Storm Behind the Silence: Detecting Cerebral Amyloid Angiopathy-Related Inflammation in a Patient with Dementia.
Journal of Brown hospital medicineProgressive Bilateral Sensorineural Hearing Loss and Ataxia Caused by Superficial Siderosis Secondary to Chronic Spontaneous Intracranial Hypotension.
CureusSevere symptomatic amyloid-related imaging abnormalities in Alzheimer's disease: Two case reports and systematic review of reported cases.
Journal of Alzheimer's disease : JAD18F-MK-6240 uptake in cortical tau and hemorrhagic lesions in a case of Alzheimer's disease with possible crossed aphasia.
eNeurologicalSciBiopsy-Confirmed Cerebral Amyloid Angiopathy-Related Inflammation With Absence of Cerebral Microbleeds on T2* and Detectable Only by Susceptibility-Weighted Imaging.
CureusPathological findings from surgical specimens and long-term outcome in cerebral amyloid angiopathy patients with intracerebral hemorrhage.
Scientific reportsCognitive Age Delta as a Marker of Healthy and Pathological Cognitive Aging: The Role of Lifestyle, Cognitive Reserve, and Vascular Risk.
Journal of clinical medicineIatrogenic cerebral amyloid angiopathy 30 years after childhood cadaveric dura mater transplantation: a case report.
BMC neurologyDuropathies: A Narrative Overview of a Neglected Concept-Part One: Anatomical, Embryological, and Pathophysiological Elements.
NeuroSciProfiles of blood-brain barrier and neurodegeneration markers in cerebrospinal fluid of patients with cerebral amyloid angiopathy.
Alzheimer's & dementia (Amsterdam, Netherlands)Electroconvulsive Therapy for Refractory Anxiety and Depression in Superficial Siderosis: A Case Report.
Clinical neuropharmacologyWhite Matter Free Water and PSMD as Neuroimaging Biomarkers of Cerebral Amyloid Angiopathy Severity.
NeurologyIatrogenic cerebral amyloid angiopathy with inflammatory pathogenesis: A case report and literature review.
Surgical neurology internationalNeuroimaging Markers Associated With Recurrent Stroke in Intracerebral Hemorrhage and Atrial Fibrillation: Secondary Analysis of PRESTIGE-AF.
NeurologyHigher Burden of Cerebral Small Vessel Disease Is Associated With Risk of Incident Stroke in Community-Dwelling Individuals.
Journal of the American Heart Association[Ataxia, deafness and diplopia].
La Revue de medecine interneTo select or not to select: evaluating the effectiveness of SeLECT score in predicting the risk of post-stroke seizures: a systematic review.
Epilepsy & behavior : E&BIn Vivo Imaging of Blood-Brain Barrier Leakage Using a Contrast Agent in Patients With Cerebral Amyloid Angiopathy: An Exploratory Study.
NeurologyCerebral amyloid angiopathy-related inflammation superimposed on alpha-synucleinopathies: a case report and literature review.
Journal of neuroimmunologyDeterminants of cerebral amyloid angiopathy progression: a multicenter retrospective study.
Journal of neurologyMicrovascular 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 SocietyCerebrospinal Fluid Biomarkers Profiling in Cerebral Amyloid Angiopathy and Relationship With Disease Phenotypes.
Journal of the American Heart AssociationSuperficial siderosis in Marfan syndrome.
Practical neurologySafety and effectiveness of lecanemab in Chinese patients with early Alzheimer's disease: Evidence from a multidimensional real-world study.
Chinese medical journalAmyloid-related Imaging Abnormalities (ARIA) in the Context of Alzheimer's Disease and Amyloid-targeting Therapies: An Introduction for Advanced Practice Providers.
Drugs & agingIatrogenic 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 SocietyMultimarker Cerebral Small Vessel Disease Score and Risk of Incident Dementia in the Framingham Heart Study.
NeurologyThe role of brain MR and FDG-PET in the diagnosis of neurodegenerative disease.
European radiologyWhen Blood Leaves a Trace: Diagnosing Superficial Siderosis in a Geriatric Patient.
CureusSATURN MRI: study protocol for the statin use in intracerebral hemorrhage patients MRI ancillary study.
TrialsPrevalent Cardiovascular Disease and Atrial Fibrillation in Relation to Cerebral Small Vessel Disease Burden.
Brain sciencesCoexisting Subdural Hematoma in Cerebral Amyloid Angiopathy: A Case Series.
Neurology internationalMulti-marker cerebral small vessel disease score and risk of incident depression: The Framingham Heart Study.
Journal of affective disordersPrevalence and risk factors of cerebral microhemorrhages and superficial siderosis in cognitively unimpaired older adults: analysis from the CHARIOT-PRO SubStudy.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationDiabetic Ketosis Complicated with Superficial Siderosis.
Neurology IndiaIntraventricular cavernous hemangioma hemorrhage leads to Superficial Siderosis of the Central Nervous System.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaCerebral Amyloid Angiopathy-Related Inflammation: Clinical Characteristics and Treatment Experience.
Clinical interventions in agingSpinal Myxopapillary Ependymoma Presenting With Isolated Intracranial Hypertension: A Case Report.
CureusMRI Findings of Intracranial Hemorrhages in ARCADIA-MRI: An Ancillary Study to the ARCADIA Trial.
AJNR. American journal of neuroradiologyAccelerated MRI Sequences for Intracranial Hemorrhage Screening.
Investigative radiologyCentral nervous system superficial siderosis: A case report and literature review.
MedicineCerebral 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.
CureusAudiological Progression and Treatment Outcomes in Superficial Siderosis: A 10-Year Retrospective Review of 46 Patients.
Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and NeurotologyClinical Management of Cerebral Amyloid Angiopathy.
Journal of clinical medicineOutcomes of CT-Guided Targeted Epidural Patching for Lateral Dural Tears in Spontaneous Intracranial Hypotension: A Multicenter Retrospective Cohort Study.
AJNR. American journal of neuroradiologyMonitoring of amyloid related imaging abnormalities: SWI vs T2*-GRE.
The journal of prevention of Alzheimer's diseasePrediction Model to Optimize Long-Term Antithrombotic Therapy Using Covert Vascular Brain Injury and Clinical Features.
Stroke"Real-world" eligibility for anti-amyloid treatment in a tertiary memory clinic setting.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationMRI protocols and sequences for amyloid-related imaging abnormalities monitoring in Alzheimer's disease patients treated with monoclonal antibodies.
Current opinion in neurologyVascular β-amyloid potentially colocalises with phosphorylated tau in cerebral amyloid angiopathy.
Stroke and vascular neurologyClinical Features and Diagnosis of Spontaneous Intracranial Hypotension.
Continuum (Minneapolis, Minn.)Cerebral Amyloid Angiopathy: A Case Report.
CureusIatrogenic Cerebral Amyloid Angiopathy After Childhood Brain Surgery: Novel Findings of MRI and CT.
Neurology internationalCochlear Implantation: A Treatment Approach of Hearing Loss in Superficial Siderosis.
The journal of international advanced otologyLecanemab Treatment in a Specialty Memory Clinic.
JAMA neurologyCerebral Amyloid Angiopathy and Downstream Alzheimer Disease Plasma Biomarkers.
JAMA network openCSF-venous fistulae-An underrecognized cause of superficial siderosis.
Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciencesAdded Value of Cerebral Small Vascular Disease Burden Based On MRI for Prediction Poor Outcome of Intracerebral Hemorrhage.
Clinical neuroradiologyRe-evaluation of the efficacy and safety of anti-Aβ monoclonal antibodies (lecanemab/donanemab) in the treatment of early Alzheimer's disease.
Frontiers in pharmacologyNew-Onset Progressive Ataxia in a 66-Year-Old Woman with Longstanding Parkinson's Disease: A Case Report.
Case reports in neurologyOne-Year Radiologic Progression in Sporadic and Hereditary Cerebral Amyloid Angiopathy.
NeurologySeizures in Cerebral Amyloid Angiopathy: A Systematic Review and Meta-Analysis.
Neurology. Clinical practiceDonanemab: Appropriate use recommendations.
The journal of prevention of Alzheimer's diseaseCerebral Amyloid Angiopathy: Clinical Presentation, Sequelae and Neuroimaging Features-An Update.
BiomedicinesSafety 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. NeurologyMaximizing 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 NeuroTherapeuticsIncidence of Amyloid-Related Imaging Abnormalities in Phase III Clinical Trials of Anti-Amyloid-β Immunotherapy: An Updated Meta-Analysis.
NeurologyClinical and Neuroradiological Manifestations of Cerebral Amyloid Angiopathy: A Closer Look into the Natural History of a Frequent Disease.
Journal of clinical medicineSpinal myxopapillary ependymoma presenting with intracranial hypertension.
Practical neurologyAmyloid-Related Imaging Abnormalities With Donanemab in Early Symptomatic Alzheimer Disease: Secondary Analysis of the TRAILBLAZER-ALZ and ALZ 2 Randomized Clinical Trials.
JAMA neurologyPrevalence of infratentorial superficial siderosis in a large general population sample from the UK Biobank.
Journal of neurologyLong-Term Cognitive Decline After Subarachnoid Hemorrhage: Pathophysiology, Management, and Future Directions.
StrokeCerebrospinal Fluid Beta-Amyloid Concentration and Clinical and Radiographic Manifestations of Cerebral Amyloid Angiopathy.
Journal of the American Heart AssociationExplainable models for predicting long-term outcomes in patients with spontaneous intracerebral haemorrhage: a retrospective cohort study.
Stroke and vascular neurologyA Case of Cerebral Amyloid Angiopathy With Recurrent Hemorrhagic and Ischemic Strokes Under Direct Oral Anticoagulant Therapy for Atrial Fibrillation.
Cureus[Clinical characteristics and prognosis of type 1 infratentorial superficial siderosis].
Zhonghua yi xue za zhiTeaching NeuroImage: Superficial Siderosis in Marfan Syndrome.
NeurologyCerebral Amyloid Angiopathy in Alzheimer Disease: A Comparison Between Different Versions of the Boston Criteria.
NeurologyClinical diagnosis of cerebral amyloid angiopathy related hemorrhage in China: Simplified Edinburgh criteria and Boston criteria version 2.0.
European stroke journalAmyloid-related imaging abnormalities: manifestations, metrics and mechanisms.
Nature reviews. NeurologyInfratentorial superficial siderosis due to a spinal dural tear.
Practical neurologyIncidental asymptomatic spinal cerebrospinal fluid leaks: single-center experience, and a presentation of seven cases.
Journal of neurointerventional surgeryInsights into the transcriptomic heterogeneity of brain endothelial cells in normal aging and Alzheimer's disease.
Neural regeneration researchCognitive Impairment in Cerebral Amyloid Angiopathy: A Single-Center Prospective Cohort Study.
Journal of clinical medicineHigher burden of cerebral small vessel disease is associated with risk of incident stroke in community dwelling individuals.
medRxiv : the preprint server for health sciencesAmyloid-Related Imaging Abnormalities (ARIA) in Clinical Trials of Gantenerumab in Early Alzheimer Disease.
JAMA neurologyMRI-Based Prediction of Macrovascular Causes of Intracerebral Hemorrhage: The MACRO Score.
NeurologySpinal subarachnoid hematoma involving a thoracic ventral dural defect: illustrative case.
Journal of neurosurgery. Case lessonsSuperficial Siderosis: A Case Report of Underdiagnosed Disorder.
CureusAcute to Chronic Variants in the Imaging Spectrum of Superficial Siderosis: Case Series and Literature Review.
CureusYAP1 fusion-positive ependymoma presenting in an adult with a pigmented phenotype and association with superficial siderosis.
Free neuropathologyVentral Thoracic Cerebrospinal Fluid Leak Repair in Spontaneous Intracranial Hypotension Complicated by Superficial Siderosis: 2-Dimensional Operative Video.
Operative neurosurgery (Hagerstown, Md.)[A case of young onset cerebral amyloid angiopathy associated with dural grafting].
Rinsho shinkeigaku = Clinical neurologyFrom Spinal Ependymoma to Superficial Siderosis: A Bottom to Top Cause of Progressive Neurological Deterioration.
The NeurohospitalistDuropathy as a rare motor neuron disease mimic: from bibrachial amyotrophy to infratentorial superficial siderosis.
BMC neurologyBrain 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 AmyloidosisAmyloid-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 radiologyClinical and neuroimaging precursors in cerebral amyloid angiopathy: impact of the Boston criteria version 2.0.
European journal of neurologyA 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 internationalApplication of artificial intelligence-based magnetic resonance imaging in diagnosis of cerebral small vessel disease.
CNS neuroscience & therapeuticsAssociation of enlarged perivascular spaces with cognitive function in dementia-free older adults: A population-based study.
Alzheimer's & dementia (Amsterdam, Netherlands)Tumefactive cerebral amyloid angiopathy - related inflammation.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyThe hidden link between late-onset seizures and cerebral amyloid angiopathy: A case-control study.
Epilepsia openSpontaneous intracranial hypotension - a spinal disease.
RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der NuklearmedizinOccult Amyloid-β-Related Angiitis: Neuroimaging Findings at 1.5T, 3T, and 7T MRI.
AJNR. American journal of neuroradiologyA Case of Superficial Siderosis with Elevated Anti-Ro/SSA Antibody.
NMC case report journalCerebral Amyloid Angiopathy-Related Inflammation and Biopsy-Positive Primary Angiitis of the CNS: A Comparative Study.
NeurologySuperficial Siderosis of the Central Nervous System: A Report of Two Cases With Spinal Pathology and a Review of the Literature.
CureusNon-hemorrhagic imaging markers of cerebral amyloid angiopathy in memory clinic patients.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationQuantitative electroencephalography in cerebral amyloid angiopathy.
Clinical neurophysiology : official journal of the International Federation of Clinical NeurophysiologyClinical characteristics of cerebral amyloid angiopathy and risk factors of cerebral amyloid angiopathy related intracerebral hemorrhage.
Journal of neurologyCentral Nervous System Siderosis Associated with Multiple Cerebral Aneurysms: Literature Review and Description of an Additional Case.
World neurosurgeryPathophysiology, Diagnosis, Prognosis, and Prevention of Poststroke Epilepsy: Clinical and Research Implications.
NeurologySuperficial Siderosis Causing Refractory Papilledema Following Brain Tumor Resection.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology SocietySpinal intradural pseudocyst formation in central nervous system superficial siderosis.
Brain pathology (Zurich, Switzerland)Detection of Dural Defect Localization Using 4-Dimensional Dynamic Computed Tomography Myelography for Patients with Superficial Siderosis.
World neurosurgerySex differences in histopathological markers of cerebral amyloid angiopathy and related hemorrhage.
International journal of stroke : official journal of the International Stroke SocietyHemorrhagic Transformation in Noncardioembolic Acute Ischemic Stroke: MRI Analysis From PACIFIC-STROKE.
StrokeCerebral tau pathology in cerebral amyloid angiopathy.
Brain communicationsProgressive superficial siderosis despite complete remission of intracranial hypotension symptoms following epidural patching: Case report.
HeadacheSpontaneous Intracranial Hypotension: Clinical Presentation, Diagnosis, and Treatment Strategies.
Neurologic clinicsSuperficial Siderosis After Traumatic Brain Injury: A Case Report.
CureusSuperficial small cerebellar infarcts in cerebral amyloid angiopathy on 3 T MRI: A preliminary study.
Journal of the neurological sciencesLumbar Subarachnoid-Peritoneal Shunting Deteriorates Superficial Siderosis Associated with a Dural Defect.
CureusCorrigendum: Infratentorial superficial siderosis: report of six cases and review of the literature.
Frontiers in neuroscience[Cerebral amyloid angiopathy].
Zhurnal nevrologii i psikhiatrii imeni S.S. KorsakovaCardiovascular Management in Asymptomatic (Silent) Cerebral Microbleeds and Suspected Cerebral Amyloid Angiopathy.
StrokeTemporal Ordering of Biomarkers in Dutch-Type Hereditary Cerebral Amyloid Angiopathy.
StrokeCerebral Superficial Siderosis.
Neurology IndiaInfratentorial superficial siderosis: report of six cases and review of the literature.
Frontiers in neuroscienceAdvances 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 neurologiquesThe 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 reportsSpinal Anterior Dural Dissection: Moving From Differential to Unifying Diagnosis.
NeurosurgeryArtificial Intelligence Assistive Software Tool for Automated Detection and Quantification of Amyloid-Related Imaging Abnormalities.
JAMA network openResearch landscape and trends of cerebral amyloid angiopathy: a 25-year scientometric analysis.
Frontiers in neurologyDeep learning applications in vascular dementia using neuroimaging.
Current opinion in psychiatryPost-transfusion severe headache in a patient with thalassemia with superficial siderosis of the central nervous system: a case report and literature review.
BMC neurologyGenome-Wide Association Studies of ARIA From the Aducanumab Phase 3 ENGAGE and EMERGE Studies.
NeurologySuperficial siderosis and the dura.
European journal of neurology[Cerebral Small Vessel Disease: Advances in Understanding its Pathophysiology].
Fortschritte der Neurologie-PsychiatrieLeft atrial appendage occlusion in patients with spontaneous intracerebral hemorrhage: An observational study.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke AssociationSWI versus GRE-T2*: Assessing cortical superficial siderosis in advanced cerebral amyloid angiopathy.
Revue neurologiqueRisk factors for recurrent cerebral amyloid angiopathy-related intracerebral hemorrhage.
Frontiers in neurologyThe 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 researchSurgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis.
European journal of neurologyCombined effect of cortical superficial siderosis and cerebral microbleed on short-term and long-term outcomes after intracerebral haemorrhage.
Stroke and vascular neurology[Superficial siderosis of the central nervous system. Report of one case].
Revista medica de ChileApraxia Associated With Superficial Siderosis in Cerebral Amyloid Angiopathy.
Journal of clinical neurology (Seoul, Korea)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 NeurophysiologyEtiology of Primary Cerebellar Intracerebral Hemorrhage Based on Topographic Localization.
StrokeDetection rate of brain MR and MR angiography for neuroimaging abnormality in patients with newly diagnosed left-sided infective endocarditis.
Scientific reports[Analysis of the prevalence of anticoagulant therapy in patients with cognitive disorders and cerebral amyloid angiopathy (CAA)].
Der NervenarztA superficial siderosis-related transient focal neurological episode in a patient with Marfan syndrome.
Chronic diseases and translational medicineA case report of superficial siderosis of the central nervous system and literature review.
The Journal of international medical researchSuperficial Cerebellar Siderosis and Spontaneous Intracranial Hypotension Secondary to Dural Tear.
The NeurohospitalistClinical Characteristics, Neuroimaging Markers, and Outcomes in Patients with Cerebral Amyloid Angiopathy: A Prospective Cohort Study.
Journal of clinical medicineCerebral Microbleed Patterns and Cortical Amyloid-β: The ARIC-PET Study.
StrokeCortical superficial siderosis is associated with reactive astrogliosis in cerebral amyloid angiopathy.
Journal of neuroinflammationImpact of previous statin use on first intracerebral hemorrhage in cerebral amyloid angiopathy.
Revue neurologiquePostoperative vasospasm and cerebral infarction in a patient with large pituitary adenoma and cerebral superficial siderosis.
Surgical neurology international[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 neurologyCognitive Versus Hemorrhagic Onset in Cerebral Amyloid Angiopathy: Neuroimaging Features.
Current Alzheimer researchAge-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 AssociationPreceding symptoms and temporal development of cortical superficial siderosis in cerebral amyloid angiopathy: a case report.
BMC neurologyAssociation of the Presence and Pattern of MRI Markers of Cerebral Small Vessel Disease With Recurrent Intracerebral Hemorrhage.
NeurologyMechanistic Implications of Cortical Superficial Siderosis in Patients With Mixed Location Intracerebral Hemorrhage and Cerebral Microbleeds.
NeurologyQuantitative measurement of cortical superficial siderosis in cerebral amyloid angiopathy.
NeuroImage. ClinicalIncidence and risk factors associated with seizures in cerebral amyloid angiopathy.
European journal of neurologySuperficial siderosis: comparison of two cases indicates two distinct diagnostic entities. Illustrative cases.
Journal of neurosurgery. Case lessonsCase report of iatrogenic cerebral amyloid angiopathy after exposure to Lyodura: an Australian perspective.
Frontiers in neuroscienceCortical superficial siderosis, hematoma volume, and outcomes after intracerebral hemorrhage: a mediation analysis.
Frontiers in neurologyAmyloid and tau PET in cerebral amyloid angiopathy-related inflammation two case reports and literature review.
Frontiers in neurologyPhenotype and imaging features associated with APP duplications.
Alzheimer's research & therapyAssociações
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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.
- [Amyloid β-related angiitis (ABRA) with dural contrast enhancement].
- Quantitative evaluation of iron chelator effects on central motor and sensory tracts in superficial siderosis.
- Lifelong trajectories of modifiable vascular risk factors and relation to cerebral small vessel disease in the Framingham Heart Study.
- Cerebral microbleed patterns and the risk of incident dementia in elderly adults: The ARIC study.
- Prevalence of vestibulo-ocular reflex dysfunction in people with neurological disorders: a systematic review and meta-analysis.
- Giant cauda equina neuroendocrine tumor with superficial siderosis: illustrative case.
- Posterior confluent white matter hyperintensities and intracerebral hemorrhage recurrence risk in patients with cerebral amyloid angiopathy.
- Duropathies as Unifying Concept-Part Two: A Narrative Overview of Clinical and Neuroradiological Features.
- Abducens nerve palsy as a false localising sign in spontaneous intracranial hypotension.
- Interim analysis of all-case post-marketing surveillance study in Japan: lecanemab in patients with early Alzheimer's disease.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:247245(Orphanet)
- MONDO:0016594(MONDO)
- GARD:9484(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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
