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INTRODUCTION: Periarteritis nodosa (PAN) is a vasculitis affecting medium-vessel and may be associated with myelodysplastic syndrome. This association needs a simultaneous treatment of the vascular and the hematological disease. However limited data are available on the benefit of hematological treatment, and in particular allogeneic stem cell transplantation, in this situation. CASE REPORT: A 32-year-old patient with refractory periarteritis nodosa and simultaneous myelodysplastic syndrome, was treated with chemotherapy followed by hematopoietic stem cell allograft. The symptoms relating to PAN improved, allowing to decrease the dose of prednisone down to 5mg/d. However, a hematological relapse occurred two months later leading to the patient's death. CONCLUSION: Hematopoietic stem cell allograft may represent a therapeutic option in the management of severe or refractory autoimmune diseases when the hematological indication is retained.
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Calcinose , Lipoma , Osso e Ossos , Calcificação Fisiológica , Calcinose/diagnóstico por imagem , HumanosRESUMO
A 34-year-old man presented with fever, palpitations, maculopapular rash, pharyngitis, left cheilitis, and bilateral gonalgia. High-sensitivity troponin I concentration was 4,900 ng/l. Transthoracic echocardiogram revealed reduced global longitudinal strain. Cardiac magnetic resonance imaging showed acute myocarditis. Adult-onset Still's disease was diagnosed, and treatment with intravenous corticosteroids and tocilizumab was initiated. (Level of Difficulty: Beginner.).
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Immune checkpoint inhibitors (ICIs) have improved the management and the prognosis of patients with cancer but are associated with an increased risk of toxicities that can affect every organ. ICI-associated myocarditis has a low incidence (< 1%) but a high fatality rate (30%-50%). Herein we report a patient treated with ICI admitted for suspicion of myocarditis. Cardiac magnetic resonance imaging (cMRI) was normal. An endomyocardial biopsy (EMB) was in favour of ICI-related myocarditis with cardiac lymphohistiocytic infiltrate. This case highlights the role of a systematic evaluation with electrocardiography and troponin and the potential value of EMB in patients without a suggestive cMRI.
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Biópsia/métodos , Diagnóstico Precoce , Inibidores de Checkpoint Imunológico/efeitos adversos , Neoplasias Pulmonares/tratamento farmacológico , Imagem Cinética por Ressonância Magnética/métodos , Miocardite/diagnóstico , Miocárdio/patologia , Biomarcadores/sangue , Eletrocardiografia , Feminino , Humanos , Inibidores de Checkpoint Imunológico/administração & dosagem , Infusões Intravenosas , Ipilimumab/administração & dosagem , Ipilimumab/efeitos adversos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/secundário , Pessoa de Meia-Idade , Miocardite/sangue , Miocardite/induzido quimicamente , Metástase Neoplásica , Nivolumabe/administração & dosagem , Nivolumabe/efeitos adversos , Troponina/sangueRESUMO
Chylothorax is a rare cause of pleural effusion, secondary to accumulation of lymph in the pleural space. Diagnosis is based on the triglyceride and cholesterol content of pleural fluid obtained with thoracentesis. Because the lymphatic system plays an essential role in fat absorption and immune response, lymphatic leak associated with chylothorax may cause life-threatening malnutrition and immunodeficiency. Chylothorax is usually described as traumatic or nontraumatic. The main cause of chylothorax is traumatic, typically postsurgical, secondary to iatrogenic direct puncture of the thoracic duct during thoracic surgery. Causes of nontraumatic chylothorax include a wide range of differential diagnoses. Lymphoma and thoracic malignancies are the most common causes and are responsible for chylothorax by extrinsic compression or invasion of the thoracic duct. Other rare causes include primary and secondary diffuse lymphatic diseases, responsible for chylothorax by lymphatic vessel wall dysfunction. Imaging the lymphatic system remains a challenge in the days of modern imaging. Nonenhanced MR lymphography is a noninvasive technique based on heavily T2-weighted sequences, thus enabling visualization of the lymphatic circulation. This technique allows diagnosis and differential diagnosis, evaluation of disease severity, and guidance of therapeutic management in nontraumatic chylothorax. Furthermore, it may offer radiologic classification of primary lymphatic diseases on the basis of morphologic features of lymphatic vessels. The authors describe the anatomy and physiology of the thoracic lymphatic system, present the technique of nonenhanced MR lymphography, and discuss pathophysiologic mechanisms and imaging features in different causes of nontraumatic chylothorax. ©RSNA, 2020.
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Quilotórax/diagnóstico por imagem , Quilotórax/etiologia , Doenças Linfáticas/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Diagnóstico Diferencial , Humanos , Imageamento TridimensionalRESUMO
OBJECTIVE: Turner syndrome (TS) is a rare disorder affecting 1/2500 female newborn. Aortic dilatation (AD) and aortic dissection represent a major concern in TS. The aims of our study were to describe the aortic root growth, potential aortic dilatation (AD) risk factors and cardiovascular outcomes in a cohort of patients with TS. METHODS: Among 204 adult patients included, 197 were studied using a standardized 1.5 Tesla MRI protocol. AD was defined as an aortic diameter ≥20 mm/m2 at the Valsalva sinuses and/or at the ascending aorta, when indexed to body surface area. RESULTS: At baseline, AD was present in 81/197 (41.1%) and 32/197 (16.2%) of patients, at the levels of Valsalva and ascending aorta, respectively. The aortic Valsalva diameter was larger in patients treated for thyroiditis (P < 0.001). Potential risk factors of AD were aging (P < 0.001) and the presence of bicuspid aortic valve (BAV) (P = 0.002). The hazard ratio (HR) of AD occurrence in the presence of BAV was 2.2 (95% CI: 1.33-3.71). After a median follow-up period of 5.1 years (n = 143), AD was present in 58/143 (40.6%) and 25/143 (17.5%) of patients at the levels of Valsalva and ascending aorta, respectively. The median aortic growth of the Valsalva sinuses remained stable. At the ascending aorta, it increased by 0.14 ± 0.61 mm/year. Only one aortic-related death was observed. CONCLUSION: AD is common in adult patients with TS. However, our results are rather reassuring, as the median aortic diameters remained stable after 5.1 years and few aortic events were observed.
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Doenças da Aorta/epidemiologia , Síndrome de Turner/epidemiologia , Adulto , Aorta/diagnóstico por imagem , Aorta/patologia , Doenças da Aorta/complicações , Doenças da Aorta/diagnóstico , Valva Aórtica/anormalidades , Valva Aórtica/patologia , Doença da Válvula Aórtica Bicúspide , Estudos de Coortes , Dilatação Patológica/complicações , Dilatação Patológica/diagnóstico , Dilatação Patológica/epidemiologia , Progressão da Doença , Feminino , França/epidemiologia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/epidemiologia , Doenças das Valvas Cardíacas/patologia , Humanos , Masculino , Prevalência , Síndrome de Turner/complicações , Adulto JovemRESUMO
PURPOSE: Arterial spin labeling (ASL) is a non-invasive tool measuring cerebral blood flow (CBF) and is useful to assess acute neurological deficit. While acute stroke presents as hypoperfused vascular territory, epileptic activity causes cortical hyperperfusion. Other neurological conditions exhibit hyperperfusion, like migraine or secondary "luxury perfusion" in strokes. Our objectives were to evaluate the usefulness and potential specificities of ASL in acute seizure and correlate it with electroencephalogram. MATERIALS AND METHODS: Amongst a cohort of patients with neurological deficit, addressed for suspicion of stroke, we retrospectively reviewed 25 consecutive patients with seizures who underwent magnetic resonance imaging (MRI) with ASL and electroencephalography (EEG). We compared them with a control group of patients with migraine and stroke secondary re-perfusion, exhibiting ASL hyperperfusion. RESULTS: Lateralized cortical hyperperfusion (high relative CBF) was observed in all patients. Good topographic correlation with EEG was found in 18 patients (72%). Eight (32%) had hyperperfusion of ipsilateral pulvinar, 5 (20%) had hyperperfused contralateral cerebellar hemisphere, 16 (64%) presented diffusion abnormalities and 20 (80%) had underlying epileptogenic lesions. Pulvinar hyperperfusion was not observed in the control group, nor were diffusion abnormalities in migrainous patients. Contralateral cerebellar hyperperfusion was observed in two migrainous patient, without associated pulvinar activation, whereas all patients with cerebellar hyperperfusion in the study group had associated pulvinar activation. CONCLUSIONS: Elevated CBF can be observed in the epileptogenic zone, ipsilateral pulvinar and contralateral cerebellum (diaschisis) in seizure. These abnormalities seem specific when compared with other causes of hyperperfusion. Arterial spin labeling can be highly effective in the differential diagnosis of strokes.
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Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Angiografia por Ressonância Magnética/métodos , Convulsões/diagnóstico por imagem , Convulsões/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Encéfalo/irrigação sanguínea , Eletroencefalografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Marcadores de Spin , Adulto JovemRESUMO
Calcific tendinitis is a common disorder in adults. Although rare, humerus involvement associated with pectoralis major tendon calcification migration has previously been reported in studies that focus on its CT and MRI appearance. We present four cases of intraosseous migration of pectoralis major tendon calcification and their ultrasound features. Calcification migration into osseous structures is associated with cortical erosions, intraosseous calcifications, and bone marrow edema. These findings may be misleading on MRI, where the significant bone marrow and surrounding tissue inflammation may raise concerns regarding neoplasia and lead to unnecessary invasive biopsy. In these cases, echography can be less alarming, enabling the identification of the calcification, its location in the pectoralis major tendon enthesis, and the continuity between the calcification and the typical cortical erosion. Ultrasound features may be diagnostic and might help avoid additional costly imaging studies or invasive biopsy.
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Doenças Ósseas/diagnóstico por imagem , Calcinose/diagnóstico por imagem , Músculos Peitorais/diagnóstico por imagem , Tendinopatia/diagnóstico por imagem , Ultrassonografia/métodos , Idoso , Doenças Ósseas/complicações , Calcinose/complicações , Progressão da Doença , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Dor/etiologia , Tendinopatia/complicaçõesRESUMO
RATIONALE AND OBJECTIVES: Diagnosis of cardiac amyloidosis (CA) on cardiac magnetic resonance (CMR) can be challenging and quantitative indexes are relevant to further characterize the myocardium. We hypothesize that the relative myocardial enhancement measured from pre and post contrast cine imaging provides diagnostic information for CA in the setting of left ventricular hypertrophy (LVH). MATERIALS AND METHODS: Patients with LVH referred to our center and control subjects with normal CMR were retrospectively included. Percentage of myocardial enhancement (percentage ME) was obtained from pre and post contrast (5 minutes) cine sequences. Post contrast myocardial T1 and LGE extent were also recorded. RESULTS: Twenty-one patients with CA, 25 patients with non-amyloid left ventricular myocardial hypertrophy (CH) and 20 controls with normal CMR were analyzed. Percentage ME was significantly higher in CA patients (200% (174-238)) than in CH patients (122% (88-151); p = 0.0001) and control patients (104% (90-149); p = 0.0001). Percentage ME was significantly correlated with the LGE extent (Rho Spearman coefficient = 0.66; p = 0.0001) and with the post contrast myocardial T1 (Rho Spearman coefficient = -0.61; p = 0.0001). With a cutoff value of 152%, the sensitivity and specificity of percentage ME for detection of CA were 90% and 80%, respectively. CONCLUSION: Percentage ME obtained from pre and post contrast cine imaging is correlated to LGE extent and myocardial T1 and may represent an additional diagnostic parameter to consider CA in patients with LVH.
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Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Meios de Contraste , Aumento da Imagem/métodos , Imagem Cinética por Ressonância Magnética/métodos , Idoso , Feminino , Coração/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e EspecificidadeAssuntos
Cisto Epidérmico/diagnóstico por imagem , Cisto Epidérmico/patologia , Imageamento por Ressonância Magnética/métodos , Carcinomatose Meníngea/diagnóstico por imagem , Carcinomatose Meníngea/patologia , Neoplasias do Sistema Nervoso Periférico/diagnóstico por imagem , Neoplasias do Sistema Nervoso Periférico/patologia , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/patologia , Idoso , Transformação Celular Neoplásica , Meios de Contraste , Evolução Fatal , HumanosRESUMO
Pituicytomas are rare low-grade glial lesions of neurohypophysis or adenohypophysis. They are often misdiagnosed as adenomas due to their similar morphology. Previous reports have underlined their nonspecific aspect. In our experience, presence of flow voids on magnetic resonance, which usually indicate hypervascularized tumors with strong arterial supply, may be a useful sign to identify pituicytomas. Unlike adenomas, pituicytomas are highly vascularized tumors. Second, we emphasize the strong enhancement of these tumors when compared with normal hypophysis, which may be another sign of their hypervascularization. Indeed, adenomas, on the opposite side, present less enhancement than normal hypophysis. This observation is corroborated by previous histologic studies that showed adenomas present less vascular density than normal pituitary glands. However, in large tumors, the normal hypophysis can sometimes not be individualized and thus the enhancement gradient between the tumor and normal tissue cannot be properly assessed. Due to their rarity, no previous reports had ever emphasized the specific signs that could allow us to differentiate pituicytomas from adenomas; however, it seems crucial due to the potential hemorrhagic complications of pituicytoma surgery.
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Adenoma/diagnóstico por imagem , Astrocitoma/diagnóstico por imagem , Hipófise/diagnóstico por imagem , Neoplasias Hipofisárias/diagnóstico por imagem , Adulto , Diagnóstico Diferencial , Humanos , Imageamento por Ressonância Magnética , MasculinoAssuntos
Anestesia Local/métodos , Aneurisma da Aorta Abdominal/diagnóstico por imagem , Aneurisma da Aorta Abdominal/cirurgia , Implante de Prótese Vascular/métodos , Prótese Vascular , Angiografia por Tomografia Computadorizada , Procedimentos Endovasculares , Hipnose , Idoso , Fluoroscopia , Humanos , Masculino , Desenho de PróteseRESUMO
A 30-year-old man was admitted for sudden loss of consciousness. On angio-CT, a ring-like vascular formation arising from the anterior communicating artery with a low-density center was visualized. This finding was consistent with a giant arterial aneurysm presenting with a central thrombus, also called "donut aneurysm".
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Aneurisma Roto/diagnóstico por imagem , Encéfalo/diagnóstico por imagem , Aneurisma Intracraniano/diagnóstico por imagem , Trombose Intracraniana/diagnóstico por imagem , Hemorragia Subaracnóidea/diagnóstico por imagem , Adulto , Aneurisma Roto/complicações , Aneurisma Roto/cirurgia , Edema Encefálico/etiologia , Angiografia Cerebral , Angiografia por Tomografia Computadorizada , Evolução Fatal , Humanos , Aneurisma Intracraniano/complicações , Aneurisma Intracraniano/cirurgia , Hipertensão Intracraniana/etiologia , Trombose Intracraniana/complicações , Trombose Intracraniana/cirurgia , Masculino , Procedimentos Neurocirúrgicos , Ruptura Espontânea , Hemorragia Subaracnóidea/etiologia , Hemorragia Subaracnóidea/cirurgia , Tomografia Computadorizada por Raios X , Procedimentos Cirúrgicos VascularesRESUMO
We report the case of a calcified angiomyolipoma of the kidney, fortuitously discovered on a computed tomography scan of the abdomen. Because of the presence of intratumoral calcification and macroscopic fat, renal cell carcinoma could not be ruled out by imaging. Biopsy of the mass was performed, and pathological study confirmed the diagnosis of angiomyolipoma. Biopsy of renal masses containing macroscopic fat and calcification may be considered before nephrectomy.