ABSTRACT Purpose: This study aimed to analyze the association between magnetic resonance imaging apparent diffusion coefficient map value and histopathological differentiation in patients who underwent eye enucleation due to retinoblastomas. Methods: An observational chart review study of patients with retinoblastoma that had histopathology of the lesion and orbit magnetic resonance imaging with apparent diffusion coefficient analysis at Hospital de Clínicas de Porto Alegre between November 2013 and November 2016 was performed. The histopathology was reviewed after enucleation. To analyze the difference in apparent diffusion coefficient values between the two major histopathological prognostic groups, Student’s t-test was used for the two groups. All statistical analyses were performed using SPSS version 19.0 for Microsoft Windows (SPSS, Inc., Chicago, IL, USA). Our institutional review board approved this retrospective study without obtaining informed consent. Results: Thirteen children were evaluated, and only eight underwent eye enucleation and were included in the analysis. The others were treated with photocoagulation, embolization, radiotherapy, and chemotherapy and were excluded due to the lack of histopathological results. When compared with histopathology, magnetic resonance imaging demonstrated 100% accuracy in retinoblastoma diagnosis. Optic nerve invasion detection on magnetic resonance imaging showed a 66.6% sensitivity and 80.0% specificity. Positive and negative predictive values were 66.6% and 80.0%, respectively, with an accuracy of 75%. In addition, the mean apparent diffusion coefficient of the eight eyes was 0.615 × 103 mm2/s. The mean apparent diffusion coefficient value of poorly or undifferentiated retinoblastoma and differentiated tumors were 0.520 × 103 mm2/s and 0.774 × 103 mm2/s, respectively. Conclusion: This study revealed that magnetic resonance imaging is useful in the diagnosis of retinoblastoma and detection of optic nerve infiltration, with a sensitivity of 66.6% and specificity of 80%. Our results also showed lower apparent diffusion coefficient values in poorly differentiated retinoblastomas with a mean of 0.520 × 103 mm2/s, whereas in well and moderately differentiated, the mean was 0.774 × 103 mm2/s. Purpose Methods 201 groups Students Student s ttest t test 190 19 0 19. SPSS, (SPSS Inc Inc. Chicago IL USA. USA . USA) consent Results evaluated photocoagulation embolization radiotherapy 100 666 66 6 66.6 800 80 80.0 respectively 75 75% addition 0615 615 0.61 10 mm2s mms mm2 mm 0520 520 0.52 0774 774 0.77 Conclusion infiltration 80% 20 1 66. 8 80. 7 061 61 0.6 052 52 0.5 077 77 0.7 2 06 0. 05 5 07

Abstract Background Sjogren-Larsson syndrome (SLS) is a neurocutaneous disease with an autosomal recessive inheritance, caused by mutations in the gene that encodes fatty aldehyde dehydrogenase (ALDH3A2), clinically characterized by ichthyosis, spastic diplegia, and cognitive impairment. Brain imaging plays an essential role in the diagnosis, demonstrating a nonspecific leukoencephalopathy. Data regarding brain atrophy and grey matter involvement is scarce and discordant. Objective We performed a volumetric analysis of the brain of two siblings with SLS with the aim of detecting deep grey matter nuclei, cerebellar grey matter, and brainstem volume reduction in these patients. Methods Volume data obtained from the brain magnetic resonance imaging (MRI) of the two patients using an automated segmentation software (Freesurfer) was compared with the volumes of a healthy control group. Results Statistically significant volume reduction was found in the cerebellum cortex, the brainstem, the thalamus, and the pallidum nuclei. Conclusion Volume reduction in grey matter leads to the hypothesis that SLS is not a pure leukoencephalopathy. Grey matter structures affected in the present study suggest a dysfunction more prominent in the thalamic motor pathways. SjogrenLarsson Sjogren Larsson (SLS inheritance ALDH3A2, ALDH3A2 ALDHA , ALDH A (ALDH3A2) ichthyosis diplegia impairment diagnosis leukoencephalopathy discordant nuclei MRI (MRI Freesurfer (Freesurfer group cortex thalamus pathways ALDH3A (ALDH3A2 (ALDH3A

Resumo Antecedentes A Síndrome de Sjogren-Larsson (SSL) é uma doença neurocutânea de herança autossômica recessiva, causada por mutações no gene que codifica a aldeído graxo desidrogenase (ALDH3A2), caracterizada clinicamente por ictiose, diplegia espástica e comprometimento cognitivo. A imagiologia cerebral desempenha um papel essencial no diagnóstico, demonstrando uma leucoencefalopatia inespecífica. Dados sobre atrofia cerebral e envolvimento da substância cinzenta são escassos e discordantes. Objetivo Realizamos uma análise volumétrica do cérebro de dois irmãos com SLS com o objetivo de detectar núcleos profundos de substância cinzenta, substância cerebral cinzenta e redução do volume do tronco encefálico nestes pacientes. Métodos Os dados de volume obtidos da ressonância magnética (RM) cerebral dos dois pacientes usando um software de segmentação automática (Freesurfer) foram comparados com os volumes de um grupo controle saudável. Resultados Redução de volume estatisticamente significativa foi encontrada no córtex do cerebelo, no tronco cerebral, no tálamo e nos núcleos pálidos. Conclusão A redução do volume da substância cinzenta leva à hipótese de que a SSL não é uma leucoencefalopatia pura. As estruturas da substância cinzenta afetadas no presente estudo sugerem uma disfunção mais proeminente nas vias motoras talâmicas. SjogrenLarsson Sjogren Larsson (SSL recessiva ALDH3A2, ALDH3A2 ALDHA , ALDH (ALDH3A2) ictiose cognitivo diagnóstico inespecífica discordantes RM (RM Freesurfer (Freesurfer saudável cerebelo pálidos pura talâmicas ALDH3A (ALDH3A2 (ALDH3A

Abstract Introduction Interdisciplinary investigative study of the stomatognathic-cervical complex, necessary to understand the structure and biomechanics of this system in Angle Class I and II / 2nd Division participants. Objective To evaluate alignment and position of cranial cervical structures on radiographs and their functional relationship with the stomatognathic system. Material and method Trans Oral and Profile radiographs were submitted to biomechanical analysis, considered the linear and angular measurement of Atlas and Axis through the application included in the radiogram software. Result We observed a significant difference in the angular measurements of vertical alignment between the Skull, Axis, and the Mandible (p = <0.001), and in the left joint between the Atlas and the Axis (AE: p = 0.011; SEA: p = 0.042). Among the linear measures of the distances between the Atlas and the Axis, the AOD distance presented statistics quite close to the level of significance (p = 0.0502), but above. There was no statistically significant difference in the other measures. Conclusion In this study, the alignment between the Atlas and Axis cervical vertebrae and the mandible and angles of the atlanto-occipital joints are altered in Class II / 2nd Division participants. There is no difference in the size and distance of the Atlas and the Axis between the Classes. stomatognathiccervical complex nd participants analysis software Skull <0.001, 0001 <0.001 , 0 001 <0.001) AE (AE 0.011 0011 011 SEA 0.042. 0042 0.042 . 042 0.042) 0.0502, 00502 0.0502 0502 0.0502) above atlantooccipital atlanto occipital Classes 000 <0.00 00 0.01 01 004 0.04 04 0050 0.050 050 <0.0 0.0 005 0.05 05 <0. 0. <0 <

Resumo Introdução Estudo interdisciplinar investigativo do complexo cérvico-estomatognático, necessário para compreender a estrutura e a biomecânica desse sistema em participantes Classe I e II/2ª Divisão de Angle. Objetivo Avaliar o alinhamento e a posição das estruturas crânio cervicais nas radiografias de participantes Classe I e II/2ª Divisão de Angle e sua relação funcional com o sistema estomatognático. Material e método As imagens digitais Trans Oral e Perfil de participantes com maloclusão Classe I e II/2ª Divisão de Angle foram submetidas à medição linear e angular do crânio, mandíbula, Atlas e do Áxis através do software Advantage Workstation 4.6 (AW4.6 ext. 04). Resultado Houve diferença significativa nas medidas angulares de alinhamento vertical entre o Crânio, Áxis e a Mandíbula (p = <0,001), e da articulação esquerda entre o Atlas e o Áxis (AE: p = 0,011; AAE: p = 0,042). Das medidas lineares das distâncias entre o Atlas e o Áxis, a distância AOD apresentou estatística bastante próxima do nível de significância (p=0,0502), porém acima. Não houve diferença significativamente estatística nas demais medidas avaliadas. Conclusão Neste estudo, o alinhamento entre as vértebras cervicais Atlas e Áxis e a mandíbula e os ângulos das articulações atlanto occipitais se mostraram alterados nos participantes Classe II/2ª Divisão de Angle. Não há diferença significativa no tamanho e distância do Atlas e do Áxis entre as Classes. cérvicoestomatognático, cérvicoestomatognático cérvico estomatognático, estomatognático cérvico-estomatognático II2ª IIª II 2ª ª 46 4 6 4. AW4.6 AW46 AW AW4 (AW4. ext 04. 04 . 04) Crânio <0,001, 0001 <0,001 , 0 001 <0,001) AE (AE 0,011 0011 011 AAE 0,042. 0042 0,042 042 0,042) p=0,0502, p00502 p=0,0502 0502 (p=0,0502) acima avaliadas estudo Classes AW4. (AW4 000 <0,00 00 0,01 01 004 0,04 p0050 p=0,050 050 (p=0,0502 (AW <0,0 0,0 p005 p=0,05 05 (p=0,050 <0, 0, p00 p=0,0 (p=0,05 <0 p0 p=0, (p=0,0 < p=0 (p=0, p= (p=0 (p=

Resumo A hipertensão intracraniana idiopática é caracterizada por aumento da pressão intracraniana, cefaleia e manifestações visuais. Apresenta fisiopatologia incerta, porém, alguns mecanismos já foram propostos, como o aumento do volume sanguíneo cerebral, o excesso de líquor por aumento da produção ou a redução da reabsorção, e mecanismos inflamatórios como fator causal ou mesmo determinando limitação na circulação do líquor. Predomina em mulheres obesas em idade reprodutiva. Os sintomas e sinais mais comuns são cefaleia diária, zumbido síncrono ao pulso, obscurecimentos visuais transitórios e papiledema com perda visual. Os principais achados em neuroimagem são: sela turca vazia, achatamento posterior do globo ocular/esclera, estenose do seio transverso bilateral ou do seio dominante, distensão do espaço liquórico perióptico com ou sem tortuosidade do nervo óptico e protrusão intraocular da cabeça do nervo óptico. A principal complicação da hipertensão intracraniana idiopática é a perda visual. Nesse contexto, o papel da neuroimagem no diagnóstico é fundamental, pois a doença pode ser revertida se devidamente reconhecida e precocemente tratada.

Abstract Idiopathic intracranial hypertension is characterized by increased intracranial pressure, headache, and visual perturbations. Although the pathophysiology of idiopathic intracranial hypertension is obscure, several mechanisms have been proposed, such as increased cerebral blood volume, excessive cerebrospinal fluid volume (due to high production or impaired resorption), and inflammatory mechanisms as a likely cause of or contributor to impaired cerebrospinal fluid circulation. It predominantly affects women of reproductive age who are overweight or obese. The most common symptoms are daily headache, synchronous pulsatile tinnitus, transient visual perturbations, and papilledema with visual loss. The main neuroimaging findings are a partially empty sella turcica; flattening of the posterior sclera; transverse sinus stenosis (bilateral or in the dominant sinus); a prominent perioptic subarachnoid space, with or without optic nerve tortuosity; and intraocular protrusion of the optic nerve head. The main complication of idiopathic intracranial hypertension is visual loss. Within this context, neuroimaging is a crucial diagnostic tool, because the pathology can be reversed if properly recognized and treated early.

Resumo A doença de von Hippel-Lindau (VHL) é uma desordem autossômica dominante monogênica com mutações na linha germinativa do antioncogene VHL, no braço curto do cromossomo três (3p25-26). Afeta 1:36.000-50.000 indivíduos, com penetrância superior a 90% aos 65 anos de idade. Embora tenha início e apresentação variáveis, com pleiotropismo mesmo entre membros da mesma família que partilham uma mutação específica, usualmente manifesta-se de início em adultos jovens e predispõe ao desenvolvimento de tumores benignos e malignos no sistema nervoso central (SNC) e órgãos viscerais. Clinicamente, o diagnóstico pode ser realizado em uma das seguintes circunstâncias: a) em pacientes com história familiar de doença de VHL e pelo menos um dos tumores característicos relacionados à síndrome (como hemangioblastomas retinianos ou do SNC, carcinoma de células renais de células claras, tumores neuroendócrinos pancreáticos e tumores do saco endolinfático); b) dois ou mais hemangioblastomas do SNC; c) um hemangioblastoma retiniano ou do SNC mais pelo menos um tumor característico visceral relacionado à síndrome, excluindo-se cistos renais e epididimários. Nesse contexto, a imagem ocupa importante papel no diagnóstico e acompanhamento desses pacientes. Este ensaio iconográfico apresenta imagens características de manifestações abdominais de tumores relacionados à doença de VHL que todos os radiologistas devem conhecer.

Abstract Von Hippel-Lindau (VHL) disease is a monogenic autosomal dominant disorder with germline mutations of the VHL anti-oncogene on the short arm of chromosome 3 (3p25-26). It affects 1:36,000-50,000 individuals, with a penetrance greater than 90% at 65 years of age. Although of variable onset and presentation, with pleiotropism even among members of the same family who share a specific mutation, VHL disease usually manifests initially in young adults. It predisposes to the development of benign and malignant tumors of the central nervous system (CNS) and visceral organs. The clinical diagnosis of VHL disease can be made in the following circumstances: a) in patients with a family history of the disease and at least one of the tumors characteristic of it (e.g., retinal or CNS hemangioblastomas, clear cell renal cell carcinoma, pancreatic neuroendocrine tumors, and endolymphatic sac tumors); b) in patients with two or more CNS hemangioblastomas; c) or in patients with a retinal or CNS hemangioblastoma plus at least one visceral tumor characteristic of the disease, excluding renal and epididymal cysts. Imaging plays an important role in the diagnosis and follow-up of patients with VHL disease. This pictorial essay presents characteristic images of abdominal manifestations of VHL disease-related tumors that all radiologists should be aware of.

Resumo Ataxia é definida como uma síndrome de falta de coordenação dos músculos de movimentação voluntária. Vários fatores podem causar ataxias, as quais podem ser classificadas de acordo com a idade, tipo de evolução (crônica ou aguda), cujas lesões envolvem o cerebelo e as conexões cerebelares. Com o uso correto e apropriado da neuroimagem, particularmente da ressonância magnética, o diagnóstico pode ser relativamente direito e o manejo clínico pode ser implementado de maneira correta. O objetivo deste artigo é descrever os achados de imagem na síndrome atáxica a partir de casos recuperados do arquivo digital de um hospital terciário, com a revisão dos principais achados de imagem. Neste ensaio revisamos e discutimos os aspectos de imagem de doenças neoplásicas, malformações, doenças degenerativas e doenças hereditárias relacionadas à ataxia.

Abstract Ataxia is defined as a lack of coordination of voluntary movement, caused by a variety of factors. Ataxia can be classified by the age at onset and type (chronic or acute). The causative lesions involve the cerebellum and cerebellar connections. The correct, appropriate use of neuroimaging, particularly magnetic resonance imaging, can make the diagnosis relatively straightforward and facilitate implementation of the appropriate clinical management. The purpose of this pictorial essay is to describe the imaging findings of ataxia, based on cases obtained from the archives of a tertiary care hospital, with a review of the most important findings. We also discuss and review the imaging aspects of neoplastic diseases, malformations, degenerative diseases, and hereditary diseases related to ataxia.

Resumo Ataxia é definida como uma síndrome de falta de coordenação dos músculos de movimentação voluntária. Vários fatores podem causar ataxias, os quais podem ser classificados de acordo com a idade, tipo de evolução (crônica ou aguda), cujas lesões envolvem o cerebelo e as conexões cerebelares. Com o uso correto e apropriado da neuroimagem, particularmente da ressonância magnética, o diagnóstico pode ser relativamente preciso e o manejo clínico pode ser implementado de maneira correta. O objetivo deste artigo é descrever os achados de imagem na síndrome atáxica com base em casos recuperados do arquivo digital de um hospital terciário, com a revisão dos principais achados de imagem. Neste ensaio revisamos e discutimos os aspectos imagem de doenças infecciosas, tóxicas, vasculares e inflamatórias.

Abstract Ataxia is defined as a lack of coordination of voluntary movement, caused by a variety of factors. Ataxia can be classified by the age at onset and type (chronic or acute). The causative lesions involve the cerebellum and cerebellar connections. The correct, appropriate use of neuroimaging, particularly magnetic resonance imaging, can make the diagnosis relatively accurate and facilitate implementation of the appropriate clinical management. The purpose of this pictorial essay is to describe the imaging findings of ataxia, based on cases obtained from the archives of a tertiary care hospital, with a review of the most important findings. We also review and discuss the imaging aspects of infectious, toxic, vascular, and inflammatory diseases.

SUMMARY OBJECTIVE: To evaluate the efficacy of a training program in ultrasound-guided fine needle puncture using a cost-effective model. METHODS: We evaluated the training of 20 resident radiology physicians, based on a theoretical course and a practical simulation part with models that focused on the puncture technique of thyroid nodules. The total time to perform the procedure, the number of punctures on the model surface, and the application of a questionnaire were used to assess the performance and confidence of the resident physicians in performing the procedure. RESULTS: The training model used was easy to reproduce, inexpensive, versatile, and capable of simulating the echotexture of thyroid tissue. There was a significant reduction in the total time needed to perform the procedure with a mean of 173.7 s±91.28 s from R1 and 112.8 s±17.66 s from R2 before the course vs. 19.2 s±112.8 s and 14.3 s±9.36 s, respectively, after the course (p<0.0001); as well as the number of superficial punctures, with a mean of 2.2 punctures±0.92 from R1 and 1.5 punctures±0.32 from R2 before the course vs 1.1 punctures±0.71 and 1.0 puncture±0.0, respectively, after the course (p<0.0001). There was also a subjective improvement in the performance and confidence in performing this procedure. CONCLUSIONS: An inexpensive and easy-to-reproduce gelatin-based model enabled adequate training of resident physicians and proved capable of improving their skills and confidence in simulating the procedure, even with a short period of training.