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We report here neuroimaging findings of a 41 years-old female with molecular diagnosis of CADASIL (Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy). CADASIL, the most common form of hereditary stroke, is characterized by headaches and relapsing strokes. More than 95% of patients present mutations of NOTCH3 gene. MRI, the best neuroimaging modality to investigate CADASIL can show lacunar infarcts and less-well demarcated T2 weighted hyperintensities characteristically located in subcortical white matter. There is no specific treatment for CADASIL. Anti-platelet agents such as aspirin might help to prevent new strokes. Other symptoms, like headache, seizures, or other neuropsychiatric manifestations should be appropriately treated.

We report here neuroimaging findings of a 41 years-old female with molecular diagnosis of CADASIL (Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy). CADASIL, the most common form of hereditary stroke, is characterized by headaches and relapsing strokes. More than 95% of patients present mutations of NOTCH3 gene. MRI, the best neuroimaging modality to investigate CADASIL can show lacunar infarcts and less-well demarcated T2 weighted hyperintensities characteristically located in subcortical white matter. There is no specific treatment for CADASIL. Anti-platelet agents such as aspirin might help to prevent new strokes. Other symptoms, like headache, seizures, or other neuropsychiatric manifestations should be appropriately treated.

Resumo Contexto A síndrome de May-Thurner (SMT) é a compressão da veia ilíaca esquerda (VIE) entre a artéria ilíaca direita e o corpo vertebral associada à hipertensão venosa crônica unilateral no membro inferior esquerdo. Porém, o achado tomográfico da compressão não necessariamente se reflete em sintomas. Objetivos Avaliar o achado de compressão da veia ilíaca esquerda em tomografias realizadas por outros motivos. Métodos Angiotomografias ou tomografias computadorizadas (TCs) com fase venosa foram analisadas. Foram coletados os dados demográficos e o motivo do exame, quando presente, e foi analisada a relação do diâmetro da veia ilíaca esquerda no ponto de maior compressão com um ponto a montante. Resultados De janeiro a julho de 2016, 590 tomografias foram analisadas, sendo 357 de mulheres e 233 de homens. A compressão da VIE ocorreu em 87 (14,74%) pacientes, dos quais 74 (85,05%) eram mulheres e 13 (14,9%) homens. O diâmetro médio do ponto de maior compressão entre os pacientes que apresentavam VIE < 5 mm foi de 4,4 mm, variando de 2,67 mm a 4,97 mm. O diâmetro no ponto de maior compressão representou até metade do diâmetro na última imagem justaposta ao corpo vertebral (índice de 0,5) em 179 (30,3%) dos pacientes. Conclusões Nosso estudo sugere que a ocorrência de compressão da VIE em TC de pacientes aleatórios, sem conhecimento de insuficiência venosa crônica ou TVP em MIE, é comum. Isso mostra que o achado tomográfico de compressão não necessariamente resulta em sintomas e não deve ser a única razão para tratar um paciente.

Abstract Introduction Lymphangioma is a rare congenital vascular malformation of the head and neck region isolated from the systemic circulation. It has a benign etiology, and represents 1–3% of all orbital tumors. These hamartomas often present in the pediatric population with a slightly female predilection. They have a lymphocytic composition, and may increase in size with episodes of viral infection, causing proptosis. Discussion The management of this lesion is controversial, hardly curative, and depends on the clinical presentation. The treatment options include partial surgical resection of the major cyst, needle aspiration, surgical debulking, systemic steroids, sildenafil, intralesional injection of the sclerosing agents, and local radiotherapy. Case Report In the present report, we describe an uncommon case of lymphangioma in a 6-year-old female who was first submitted to neurosurgery for tumor resection and received sildenafil therapy later, with promising results. Conclusion The treatment of orbital lymphangiomas remains a controversial topic, and the use of sildenafil along with needle aspiration and microsurgical removal is a viable option of treatment. However, many issues, such as the ideal duration of the therapy, the dosage regimen and the recurrence rate, still remain unclear. Our case report adds promising data on this pathology, even though larger trials are needed to properly elucidate the remaining questions.

IntroductionMetabolic syndrome (MetS) is an emergent problem among patients with epilepsy. Here, we evaluate and compare the diagnostic yield and accuracy of different MetS criteria among adult patients with epilepsy to further explore the best strategy for diagnosis of MetS among patients with epilepsy.Materials and methodsNinety-five epileptic adults from a tertiary epilepsy reference center were prospectively recruited over 22 weeks in a cross-sectional study. MetS was defined according to five international criteria used for the diagnosis of the condition [ATP3, American Association of Clinical Endocrinologists (AACE), International Diabetes Federation (IDF), AHA/NHLBI, and harmonized criteria]. Sensitivity, specificity, positive and negative predictive values (NPVs), and area under the receiver operating characteristic curve (ROC) curve were estimated for each criterion.ResultsIn our sample, adult patients with epilepsy showed a high prevalence of obesity, hypertension, and diabetes. However, the prevalence of MetS was significantly different according to each criterion used, ranging from 33.7%, as defined by AACE, to 49.4%, as defined by the harmonized criteria (p