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1.
Radiol Med ; 125(11): 1072-1086, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32970272

RESUMO

The restrictive cardiomyopathies constitute a heterogeneous group of myocardial diseases with a different pathogenesis and overlapping clinical presentations. Diagnosing them frequently poses a challenge. Echocardiography, electrocardiograms and laboratory tests may show non-specific changes. In this context, cardiac magnetic resonance (CMR) may play a crucial role in defining the diagnosis and guiding treatments, by offering a robust myocardial characterization based on the inherent magnetic properties of abnormal tissues, thus limiting the use of endomyocardial biopsy. In this review article, we explore the role of CMR in the assessment of a wide range of myocardial diseases causing restrictive patterns, from iron overload to cardiac amyloidosis, endomyocardial fibrosis or radiation-induced heart disease. Here, we emphasize the incremental value of novel relaxometric techniques such as T1 and T2 mapping, which may recognize different storage diseases based on the intrinsic magnetic properties of the accumulating metabolites, with or without the use of gadolinium-based contrast agents. We illustrate the importance of these CMR techniques and their great support when contrast media administration is contraindicated. Finally, we describe the useful role of cardiac computed tomography for diagnosis and management of restrictive cardiomyopathies when CMR is contraindicated.


Assuntos
Técnicas de Imagem Cardíaca/métodos , Cardiomiopatia Restritiva/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Adulto , Idoso de 80 Anos ou mais , Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatia Restritiva/classificação , Fibrose Endomiocárdica/diagnóstico por imagem , Feminino , Humanos , Sobrecarga de Ferro/diagnóstico por imagem , Doenças por Armazenamento dos Lisossomos/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Lesões por Radiação/complicações , Lesões por Radiação/diagnóstico por imagem , Sarcoidose/diagnóstico por imagem
2.
Clin Nucl Med ; 45(10): 838-839, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32796236

RESUMO

Amyloid deposition can lead to Alzheimer disease and cerebral amyloid angiopathy. Rarely, it presents as a solitary focal deposition, primary cerebral amyloidoma, which can be misinterpreted as a neoplasm because of the "tumor-like" appearances. We present the case of a 54-year-old woman where MRI revealed a T2-hyperintense mass periventricular in the white matter with moderate contrast enhancement. Pathological investigation revealed AL (lambda) amyloidoma. F-florbetapir PET/CT was used to support the diagnosis and in follow-up. This case highlights that F-florbetapir PET/CT might play a role in the diagnostic workup of patients suggestive of cerebral amyloidoma, especially in cases where biopsy is not feasible.


Assuntos
Amiloidose/diagnóstico por imagem , Compostos de Anilina , Etilenoglicóis , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Idoso , Amiloidose/patologia , Biópsia , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Substância Branca/diagnóstico por imagem , Substância Branca/patologia
3.
Rev Cardiovasc Med ; 21(2): 181-190, 2020 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-32706207

RESUMO

Heart failure with preserved ejection fraction is a very common clinical problem. Its prevalence is increasing with aging of the population. A diverse group of risk factors and etiologies comprise the HFpEF syndrome. No specific therapies have been shown to improve survival for the vast majority of HFpEF cases. Restrictive cardiomyopathies account for a significant portion of HFpEF patients and are characterized by diastolic dysfunction due to infiltration of the myocardium or ventricular hypertrophy. Many of these restrictive diseases occur in the context of myocardial infiltration by other substances such as amyloid, iron or glycogen or endomyocardial fibrosis. These infiltrative diseases usually have important clues in the clinical picture and on cardiac imaging that may allow differentiation from the usual HFpEF phenotype (that is commonly seen in the older, hypertensive patient). Noninvasive diagnosis has replaced endomyocardial biopsy for most instances in the workup of these conditions. Early recognition is important to institute specific therapies and to improve prognosis. In this review, we describe 4 major infiltrative cardiomyopathies (Cardiac Amyloidosis, Sarcoidosis, Hemochromatosis and Fabry disease), and their key imaging features.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Doença de Fabry/diagnóstico por imagem , Insuficiência Cardíaca/etiologia , Hemocromatose/diagnóstico por imagem , Volume Sistólico , Função Ventricular Esquerda , Amiloidose/complicações , Amiloidose/fisiopatologia , Amiloidose/terapia , Cardiomiopatias/complicações , Cardiomiopatias/fisiopatologia , Cardiomiopatias/terapia , Diagnóstico Diferencial , Diagnóstico Precoce , Doença de Fabry/complicações , Doença de Fabry/fisiopatologia , Doença de Fabry/terapia , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/terapia , Hemocromatose/complicações , Hemocromatose/fisiopatologia , Hemocromatose/terapia , Humanos , Valor Preditivo dos Testes , Prognóstico
4.
Clin Nucl Med ; 45(9): 705-706, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32604112

RESUMO

Amyloidosis is a disorder resulting from the deposition of fibrillary protein in the extracellular tissue and can be classified into primary, secondary, familial, and senile types. Isolated lymph node amyloidosis without any other organ involvement is very rarely seen in clinical parlance, and diagnosis remains very challenging owing to nonspecific imaging findings. We present a case of 50-year-old man with lymphadenopathy, which was later confirmed to be amyloidosis on biopsy and serum-free light chain assay with efficacious use of F-FDG PET/CT for response assessment to bortezomib, cyclophosphamide, and dexamethasone.


Assuntos
Amiloidose/diagnóstico por imagem , Amiloidose/tratamento farmacológico , Fluordesoxiglucose F18 , Linfonodos/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Amiloidose/patologia , Biópsia , Bortezomib/uso terapêutico , Ciclofosfamida/uso terapêutico , Dexametasona/uso terapêutico , Humanos , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
5.
Neurology ; 95(11): e1538-e1553, 2020 09 15.
Artigo em Inglês | MEDLINE | ID: mdl-32675080

RESUMO

OBJECTIVE: To develop and evaluate a model for staging cortical amyloid deposition using PET with high generalizability. METHODS: Three thousand twenty-seven individuals (1,763 cognitively unimpaired [CU], 658 impaired, 467 with Alzheimer disease [AD] dementia, 111 with non-AD dementia, and 28 with missing diagnosis) from 6 cohorts (European Medical Information Framework for AD, Alzheimer's and Family, Alzheimer's Biomarkers in Daily Practice, Amsterdam Dementia Cohort, Open Access Series of Imaging Studies [OASIS]-3, Alzheimer's Disease Neuroimaging Initiative [ADNI]) who underwent amyloid PET were retrospectively included; 1,049 individuals had follow-up scans. With application of dataset-specific cutoffs to global standard uptake value ratio (SUVr) values from 27 regions, single-tracer and pooled multitracer regional rankings were constructed from the frequency of abnormality across 400 CU individuals (100 per tracer). The pooled multitracer ranking was used to create a staging model consisting of 4 clusters of regions because it displayed a high and consistent correlation with each single-tracer ranking. Relationships between amyloid stage, clinical variables, and longitudinal cognitive decline were investigated. RESULTS: SUVr abnormality was most frequently observed in cingulate, followed by orbitofrontal, precuneal, and insular cortices and then the associative, temporal, and occipital regions. Abnormal amyloid levels based on binary global SUVr classification were observed in 1.0%, 5.5%, 17.9%, 90.0%, and 100.0% of individuals in stage 0 to 4, respectively. Baseline stage predicted decline in Mini-Mental State Examination (MMSE) score (ADNI: n = 867, F = 67.37, p < 0.001; OASIS: n = 475, F = 9.12, p < 0.001) and faster progression toward an MMSE score ≤25 (ADNI: n = 787, hazard ratio [HR]stage1 2.00, HRstage2 3.53, HRstage3 4.55, HRstage4 9.91, p < 0.001; OASIS: n = 469, HRstage4 4.80, p < 0.001). CONCLUSION: The pooled multitracer staging model successfully classified the level of amyloid burden in >3,000 individuals across cohorts and radiotracers and detects preglobal amyloid burden and distinct risk profiles of cognitive decline within globally amyloid-positive individuals.


Assuntos
Amiloidose/diagnóstico por imagem , Radioisótopos de Carbono , Córtex Cerebral/diagnóstico por imagem , Disfunção Cognitiva/diagnóstico por imagem , Radioisótopos de Flúor , Tomografia por Emissão de Pósitrons/métodos , Idoso , Doença de Alzheimer/diagnóstico por imagem , Doença de Alzheimer/metabolismo , Amiloidose/metabolismo , Córtex Cerebral/metabolismo , Disfunção Cognitiva/metabolismo , Estudos de Coortes , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade
6.
Lancet ; 395(10242): 1988-1997, 2020 06 27.
Artigo em Inglês | MEDLINE | ID: mdl-32593336

RESUMO

BACKGROUND: Alzheimer's disease and its complications are the leading cause of death in adults with Down syndrome. Studies have assessed Alzheimer's disease in individuals with Down syndrome, but the natural history of biomarker changes in Down syndrome has not been established. We characterised the order and timing of changes in biomarkers of Alzheimer's disease in a population of adults with Down syndrome. METHODS: We did a dual-centre cross-sectional study of adults with Down syndrome recruited through a population-based health plan in Barcelona (Spain) and through services for people with intellectual disabilities in Cambridge (UK). Cognitive impairment in participants with Down syndrome was classified with the Cambridge Cognitive Examination for Older Adults with Down Syndrome (CAMCOG-DS). Only participants with mild or moderate disability were included who had at least one of the following Alzheimer's disease measures: apolipoprotein E allele carrier status; plasma concentrations of amyloid ß peptides 1-42 and 1-40 and their ratio (Aß1-42/1-40), total tau protein, and neurofilament light chain (NFL); tau phosphorylated at threonine 181 (p-tau), and NFL in cerebrospinal fluid (CSF); and one or more of PET with 18F-fluorodeoxyglucose, PET with amyloid tracers, and MRI. Cognitively healthy euploid controls aged up to 75 years who had no biomarker abnormalities were recruited from the Sant Pau Initiative on Neurodegeneration. We used a first-order locally estimated scatterplot smoothing curve to determine the order and age at onset of the biomarker changes, and the lowest ages at the divergence with 95% CIs are also reported where appropriate. FINDINGS: Between Feb 1, 2013, and June 28, 2019 (Barcelona), and between June 1, 2009, and Dec 31, 2014 (Cambridge), we included 388 participants with Down syndrome (257 [66%] asymptomatic, 48 [12%] with prodromal Alzheimer's disease, and 83 [21%] with Alzheimer's disease dementia) and 242 euploid controls. CSF Aß1-42/1-40 and plasma NFL values changed in individuals with Down syndrome as early as the third decade of life, and amyloid PET uptake changed in the fourth decade. 18F-fluorodeoxyglucose PET and CSF p-tau changes occurred later in the fourth decade of life, followed by hippocampal atrophy and changes in cognition in the fifth decade of life. Prodromal Alzheimer's disease was diagnosed at a median age of 50·2 years (IQR 47·5-54·1), and Alzheimer's disease dementia at 53·7 years (49·5-57·2). Symptomatic Alzheimer's disease prevalence increased with age in individuals with Down syndrome, reaching 90-100% in the seventh decade of life. INTERPRETATION: Alzheimer's disease in individuals with Down syndrome has a long preclinical phase in which biomarkers follow a predictable order of changes over more than two decades. The similarities with sporadic and autosomal dominant Alzheimer's disease and the prevalence of Down syndrome make this population a suitable target for Alzheimer's disease preventive treatments. FUNDING: Instituto de Salud Carlos III, Fundació Bancaria La Caixa, Fundació La Marató de TV3, Medical Research Council, and National Institutes of Health.


Assuntos
Doença de Alzheimer/metabolismo , Biomarcadores/sangue , Síndrome de Down/complicações , Adulto , Doença de Alzheimer/líquido cefalorraquidiano , Doença de Alzheimer/diagnóstico por imagem , Doença de Alzheimer/epidemiologia , Peptídeos beta-Amiloides/metabolismo , Amiloidose/diagnóstico por imagem , Amiloidose/patologia , Apolipoproteínas E/metabolismo , Estudos de Casos e Controles , Disfunção Cognitiva/psicologia , Estudos Transversais , Síndrome de Down/epidemiologia , Síndrome de Down/mortalidade , Síndrome de Down/psicologia , Fluordesoxiglucose F18/administração & dosagem , Humanos , Imagem por Ressonância Magnética/métodos , Pessoa de Meia-Idade , Proteínas de Neurofilamentos/líquido cefalorraquidiano , Tomografia por Emissão de Pósitrons/métodos , Prevalência , Espanha/epidemiologia , Reino Unido/epidemiologia , Proteínas tau/metabolismo
8.
Curr Cardiol Rep ; 22(6): 40, 2020 05 19.
Artigo em Inglês | MEDLINE | ID: mdl-32430600

RESUMO

The gold standard for diagnosis of cardiac amyloidosis (CA) is endomyocardial biopsy showing Congo red staining followed by mass spectroscopy, but the diagnosis can also be made with high certainty by demonstration of typical cardiac imaging features along with amyloid on biopsy of another involved organ. The use of cardiac imaging techniques to detect amyloid deposits may frequently obviate the need for invasive methods in order to ascertain the presence, and potentially the type, of amyloid deposition. PURPOSE OF REVIEW: We aim to review the evidence behind the development of novel positron emission tomography (PET) radiotracers for demonstrating cardiac amyloid deposition and potentially distinguishing between light-chain (AL) or transthyretin (ATTR) cardiac amyloidosis. RECENT FINDINGS: Multiple recent studies have shown that thioflavin-analogue tracers such as18F-florbetapir, 18F-florbetaben, 18F-flutemetamol, and 11C-labeled Pittsburg Compound-B (PiB) may be able to fulfill the unmet need of elucidating the presence of amyloid deposition in the heart. Because they bind to the beta-pleated motif of the amyloid fibril due to their similarity to the thioflavin structure, they could potentially be used to image CA (Table 1). The use of PET amyloid radiotracers shows promise; however, further data is needed to define their overall accuracy and additive value to the care of patients with suspected systemic and/or cardiac amyloidosis.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiopatias/diagnóstico por imagem , Coração/diagnóstico por imagem , Tomografia por Emissão de Pósitrons/métodos , Amiloide , Cardiologia/tendências , Humanos , Pré-Albumina , Cintilografia
10.
PLoS One ; 15(5): e0232785, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32469871

RESUMO

BACKGROUND: Alzheimer's disease (AD) pathology precedes symptoms and its detection can identify at-risk individuals who may benefit from early treatment. Since the retinal nerve fiber layer (RNFL) is depleted in established AD, we tested whether its thickness can predict whether cognitively healthy (CH) individuals have a normal or pathological cerebrospinal fluid (CSF) Aß42 (A) and tau (T) ratio. METHODS: As part of an ongoing longitudinal study, we enrolled CH individuals, excluding those with cognitive impairment and significant ocular pathology. We classified the CH group into two sub-groups, normal (CH-NAT, n = 16) or pathological (CH-PAT, n = 27), using a logistic regression model from the CSF AT ratio that identified >85% of patients with a clinically probable AD diagnosis. Spectral-domain optical coherence tomography (OCT) was acquired for RNFL, ganglion cell-inner plexiform layer (GC-IPL), and macular thickness. Group differences were tested using mixed model repeated measures and a classification model derived using multiple logistic regression. RESULTS: Mean age (± standard deviation) in the CH-PAT group (n = 27; 75.2 ± 8.4 years) was similar (p = 0.50) to the CH-NAT group (n = 16; 74.1 ± 7.9 years). Mean RNFL (standard error) was thinner in the CH-PAT group by 9.8 (2.7) µm; p < 0.001. RNFL thickness classified CH-NAT vs. CH-PAT with 87% sensitivity and 56.3% specificity. CONCLUSIONS: Our retinal data predict which individuals have CSF biomarkers of AD pathology before cognitive deficits are detectable with 87% sensitivity. Such results from easy-to-acquire, objective and non-invasive measurements of the RNFL merit further study of OCT technology to monitor or screen for early AD pathology.


Assuntos
Doença de Alzheimer/genética , Peptídeos beta-Amiloides/genética , Disfunção Cognitiva/genética , Proteínas tau/genética , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/líquido cefalorraquidiano , Doença de Alzheimer/diagnóstico por imagem , Doença de Alzheimer/patologia , Peptídeos beta-Amiloides/líquido cefalorraquidiano , Amiloidose/líquido cefalorraquidiano , Amiloidose/diagnóstico por imagem , Amiloidose/genética , Amiloidose/patologia , Biomarcadores/líquido cefalorraquidiano , Disfunção Cognitiva/líquido cefalorraquidiano , Disfunção Cognitiva/diagnóstico por imagem , Disfunção Cognitiva/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fibras Nervosas/metabolismo , Fibras Nervosas/patologia , Disco Óptico/diagnóstico por imagem , Disco Óptico/metabolismo , Disco Óptico/patologia , Retina/diagnóstico por imagem , Retina/metabolismo , Retina/patologia , Células Ganglionares da Retina/metabolismo , Células Ganglionares da Retina/patologia , Tomografia de Coerência Óptica , Proteínas tau/líquido cefalorraquidiano
13.
Clin Nucl Med ; 45(5): 385-386, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32149807

RESUMO

We present a paraspinal amyloidoma found incidentally in a 77-year-old man during lymphoma workup by F-FDG PET/CT. A solitary FDG-avid paraspinal lesion was seen at T11-T12. MRI showed T2 hypointensity and enhancement. Lymphoma was considered the primary differential due to FDG uptake, but biopsy revealed nodules of extracellular acellular homogeneous material with apple-green birefringence on Congo red stain consistent with amyloidoma. Spinal amyloidoma is rare with few cases reported so far in literature.


Assuntos
Amiloidose/diagnóstico por imagem , Amiloidose/metabolismo , Fluordesoxiglucose F18/metabolismo , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/metabolismo , Idoso , Amiloidose/complicações , Transporte Biológico , Humanos , Achados Incidentais , Linfoma/complicações , Linfoma/diagnóstico por imagem , Imagem por Ressonância Magnética , Masculino
14.
Neurology ; 94(14): e1512-e1524, 2020 04 07.
Artigo em Inglês | MEDLINE | ID: mdl-32188766

RESUMO

OBJECTIVE: To examine the feasibility of using cross-sectional PET to identify cognitive decliners among ß-amyloid (Aß)-negative cognitively normal (CN) elderly adults. METHODS: We determined the highest Aß-affected region by ranking baseline and accumulation rates of florbetapir-PET regions in 355 CN elderly adults using 18F-florbetapir-PET from the Alzheimer's Disease Neuroimaging Initiative (ADNI). The banks of the superior temporal sulcus (BANKSSTS) were found as the highest Aß-affected region, and Aß positivity in this region was defined as above the lowest boundary of BANKSSTS standardized uptake value ratio of Aß+ (ADNI-defined COMPOSITE region) CN individuals. The entire CN cohort was divided as follows: stage 0, BANKSSTS-COMPOSITE-; stage 1, BANKSSTS+COMPOSITE-; and stage 2, BANKSSTS+COMPOSITE+. Linear mixed-effect (LME) models investigated subsequent longitudinal cognitive change, and 18F-flortaucipir (FTP)-PET was measured 4.8 ± 1.6 years later to track tau deposition. RESULTS: LME analysis revealed that individuals in stage 1 (n = 64) and stage 2 (n = 99) showed 2.5 (p < 0.05) and 4.8 (p < 0.001) times faster memory decline, respectively, than those in stage 0 (n = 191) over >4 years of mean follow-up. Compared to stage 0, both stage 1 (p < 0.05) and stage 2 (p < 0.001) predicted higher FTP in entorhinal cortex. CONCLUSIONS: Nominally Aß- CN individuals with high Aß in BANKSSTS are at increased risk of cognitive decline, probably showing an earlier stage of Aß deposition. Our findings may help elucidate the association between brain Aß accumulation and cognition in Aß- CN cohorts. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that in elderly CN individuals those with high PET-identified superior temporal sulcus Aß burden have an increased risk of cognitive decline.


Assuntos
Peptídeos beta-Amiloides/metabolismo , Amiloidose/diagnóstico por imagem , Amiloidose/metabolismo , Idoso , Idoso de 80 Anos ou mais , Amiloidose/psicologia , Cognição , Disfunção Cognitiva , Estudos de Coortes , Estudos Transversais , Progressão da Doença , Córtex Entorrinal/diagnóstico por imagem , Córtex Entorrinal/metabolismo , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Testes Neuropsicológicos , Tomografia por Emissão de Pósitrons , Valor Preditivo dos Testes , Lobo Temporal/diagnóstico por imagem , Lobo Temporal/metabolismo , Proteínas tau/metabolismo
15.
Cardiovasc Pathol ; 47: 107210, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32142924

RESUMO

Nonbacterial thrombotic endocarditis is a form of a thrombotic angiopathy involving the endothelial lined endocardial surfaces of the heart which includes valves and the chamber walls. Underlying etiologies for nonbacterial thrombotic endocarditis include autoimmune diseases, hypercoagulable states, in the setting of certain malignant neoplasms, and physical injury. The pathogenesis for these processes is that of primary endothelial injury resulting in a thrombotic angiopathy. We present a patient with heart failure being evaluated before hematopoietic stem cell transplantation who had previously been provided with chemotherapy and whose cardiac magnetic resonance imaging reveals findings suggestive of amyloidosis. A subsequent endomyocardial biopsy instead showed nonbacterial thrombotic endocarditis characterized by the endocardium with fibromyxoid thickening and overlying fresh fibrin. This case highlights histopathologic findings of chemotherapy-associated nonbacterial thrombotic endocarditis involving the right ventricle wall of the endocardium, therefore expanding the radiological differential in patients with cardiac magnetic resonance imaging findings suggestive of amyloidosis.


Assuntos
Amiloidose/patologia , Antineoplásicos/efeitos adversos , Endocardite não Infecciosa/induzido quimicamente , Cardiopatias/patologia , Valvas Cardíacas/efeitos dos fármacos , Trombose/induzido quimicamente , Amiloidose/diagnóstico por imagem , Biópsia , Cardiotoxicidade , Diagnóstico Diferencial , Endocardite não Infecciosa/diagnóstico por imagem , Endocardite não Infecciosa/patologia , Cardiopatias/diagnóstico por imagem , Valvas Cardíacas/diagnóstico por imagem , Valvas Cardíacas/patologia , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Trombose/diagnóstico por imagem , Trombose/patologia
16.
Clin Nucl Med ; 45(4): e206-e207, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32049735

RESUMO

Amino acid PET, including F-FDOPA, is recommended for initial characterization, delineation of tumor extent, and follow-up of gliomas because of its high diagnostic performances. F-FDOPA accumulates inside tumor cells via the L-type amino acid transporter 1 (LAT1) whose expression is increased in gliomas. We report here a case of a histopathologically proven brain amyloidoma that was first addressed for a suspected glioma. Congo red staining showed scattered extracellular deposits of amyloid and immunohistochemistry-highlighted LAT1 expression, explaining the high F-FDOPA uptake found in this lesion. This case indicates that differential diagnosis of the F-FDOPA uptake in brain lesions should include amyloidoma.


Assuntos
Amiloidose/diagnóstico por imagem , Neoplasias Encefálicas/diagnóstico por imagem , Glioma/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Idoso , Diagnóstico Diferencial , Di-Hidroxifenilalanina/análogos & derivados , Feminino , Humanos , Compostos Radiofarmacêuticos
17.
Nat Rev Cardiol ; 17(7): 413-426, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32042139

RESUMO

Systemic amyloidosis encompasses a debilitating, under-diagnosed but increasingly recognized group of disorders characterized by the extracellular deposition of misfolded proteins in one or more organs. Cardiac amyloid deposition leads to an infiltrative or restrictive cardiomyopathy and is the major contributor to poor prognosis in patients with systemic amyloidosis. In total, >30 proteins can form amyloid fibrils, but the two main types of amyloid that can infiltrate the heart are monoclonal immunoglobulin light-chain amyloid and transthyretin amyloid. Cardiac amyloidosis can be acquired in older individuals or inherited from birth. Given the nonspecific symptoms of these disorders, a high index of suspicion is paramount in making the correct diagnosis, which can involve the use of non-invasive imaging methods such as echocardiography, bone scintigraphy and cardiovascular MRI. In the past decade, the use of cardiovascular MRI with tissue characterization and bone scintigraphy to diagnose cardiac amyloidosis has revolutionized our understanding of the disease, leading to changes in patient care. However, a need remains for improved awareness and expertise, and greater clinical suspicion, because the initial clues provided by electrocardiography and echocardiography might not be typical. With specific treatments now available, timely diagnosis of cardiac amyloidosis is more important than ever. In this Review, we discuss the current and novel approaches for the diagnostic imaging of cardiac amyloidosis.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiopatias/diagnóstico por imagem , Ecocardiografia , Humanos , Imagem por Ressonância Magnética , Cintilografia
18.
J Am Coll Cardiol ; 75(4): 380-390, 2020 02 04.
Artigo em Inglês | MEDLINE | ID: mdl-32000949

RESUMO

BACKGROUND: It remains unknown whether the noninvasive evaluation of the degree of amyloid deposition in the myocardium can predict the prognosis of patients with light chain (AL) cardiac amyloidosis. OBJECTIVES: The purpose of this study was to demonstrate that 11C-Pittsburgh B compound positron emission tomography (11C-PiB PET) is useful for prognostication of AL cardiac amyloidosis by noninvasively imaging the myocardial AL amyloid deposition. METHODS: This study consecutively enrolled 41 chemotherapy-naïve AL cardiac amyloidosis patients. The amyloid deposit was quantitatively assessed with amyloid P immunohistochemistry in endomyocardial biopsy specimens and was compared with the degree of myocardial 11C-PiB uptake on PET. The primary endpoint was a composite of all-cause death, heart transplantation, and acute decompensated heart failure. RESULTS: The degree of myocardial 11C-PiB PET uptake was significantly higher in the cardiac amyloidosis patients compared with normal subjects and correlated well with the degree of amyloid deposit on histology (R2 = 0.343, p < 0.001). During follow-up (median: 423 days, interquartile range: 93 to 1,222 days), 24 patients experienced the primary endpoint. When the cardiac amyloidosis patients were divided into tertiles by the degree of myocardial 11C-PiB PET uptake, patients with the highest PiB uptake experienced the worst clinical event-free survival (log-rank p = 0.014). The degree of myocardial PiB PET uptake was a significant predictor of clinical outcome on multivariate Cox regression analysis (adjusted hazard ratio: 1.185; 95% confidence interval: 1.054 to 1.332; p = 0.005). CONCLUSIONS: These proof-of-concept results show that noninvasive evaluation of myocardial amyloid load by 11C-PiB PET reflects the degree of amyloid deposit and is an independent predictor of clinical outcome in AL cardiac amyloidosis patients.


Assuntos
Amiloidose/diagnóstico por imagem , Coração/diagnóstico por imagem , Tomografia por Emissão de Pósitrons , Idoso , Compostos de Anilina , Biópsia , Feminino , Insuficiência Cardíaca/diagnóstico por imagem , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Prognóstico , Modelos de Riscos Proporcionais , Estudos Prospectivos , Tiazóis
19.
J Clin Ultrasound ; 48(3): 168-173, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32003472

RESUMO

We describe the case of a 41-year-old woman with primary Sjögren's syndrome (SS) who presented multiple recurrences of breast amyloidosis. Each recurrence of breast amyloidosis showed different sonographic features, potentially mimicking malignancy. We briefly discuss the possible cause of this variability in imaging features based on the radiologic-histologic correlation.


Assuntos
Amiloidose/complicações , Amiloidose/diagnóstico por imagem , Doenças Mamárias/complicações , Doenças Mamárias/diagnóstico por imagem , Síndrome de Sjogren/complicações , Adulto , Amiloidose/patologia , Amiloidose/cirurgia , Doenças Mamárias/patologia , Doenças Mamárias/cirurgia , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Recidiva , Ultrassonografia/métodos
20.
Medicine (Baltimore) ; 99(6): e18978, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32028407

RESUMO

RATIONALE: AA amyloidosis (AA) is caused by a wide variety of inflammatory states, but is infrequently associated with Castleman disease (CD). CD describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. CD can present with a solitary enlarged lymph node (unicentric CD, UCD) or with multicentric lymphadenopathy (MCD), constitutional symptoms, cytopenias, and multiple organ dysfunction due to an interleukin-6 driven cytokine storm. PATIENT CONCERNS: We are reporting a case of a 26-year-old woman with no significant past medical history who presented with a 3-month history of fatigue and an unintentional 20-pound weight loss. DIAGNOSIS: A CT-scan of the abdomen and pelvis revealed hepatosplenomegaly and a mesenteric mass. Congo Red staining from a liver biopsy showed apple-green birefringence and serum markers were suggestive of an inflammatory process. Post-excision examination of the resected mass revealed a reactive lymph node with follicular hyperplasia with kappa and lambda stains showing polyclonal plasmacytosis consistent with CD. INTERVENTIONS: The patient underwent surgery to remove the affected lymph node. OUTCOMES: IL-6, anemia, leukocytosis, and thrombocytosis resolved or normalized 2 weeks after resection; creatinine normalized 9 months postsurgery. Twenty two months post-surgery her IFN-γ normalized, her fatigue resolved, her proteinuria was reduced by >90% and she had returned to her baseline weight. LESSONS: Our case and literature review suggest that patients presenting with UCD or MCD along with organ failure should prompt consideration of concurrent AA amyloidosis.


Assuntos
Amiloidose/etiologia , Hiperplasia do Linfonodo Gigante/complicações , Doenças Peritoneais/etiologia , Adulto , Amiloidose/sangue , Amiloidose/diagnóstico , Amiloidose/diagnóstico por imagem , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/diagnóstico , Fadiga/etiologia , Feminino , Humanos , Doenças Peritoneais/sangue , Doenças Peritoneais/diagnóstico , Doenças Peritoneais/diagnóstico por imagem , Proteína Amiloide A Sérica/análise , Tomografia Computadorizada por Raios X , Perda de Peso
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