Evaluation of glial tumors: correlation between magnetic resonance imaging and histopathological analysis.

Objective: To determine the correlation of conventional and diffusion-weighted imaging findings on magnetic resonance imaging (MRI) of the brain, based on Visually AcceSAble Rembrandt Images (VASARI) criteria, with the histopathological grading of gliomas: low-grade or high-grade. Materials and Methods: Preoperative MRI scans of 178 patients with brain gliomas and pathological confirmation were rated by two neuroradiologists for tumor size, location, and tumor morphology, using a standardized imaging feature set based on the VASARI criteria. Results: In the univariate analysis, more than half of the MRI characteristics evaluated showed a significant association with the tumor grade. The characteristics most significantly associated with the tumor grade were hemorrhage; restricted diffusion; pial invasion; enhancement; and a non-contrast-enhancing tumor crossing the midline. In a multivariable regression model, the presence of enhancement and hemorrhage maintained a significant association with high tumor grade. The absence of contrast enhancement and restricted diffusion were associated with the presence of an isocitrate dehydrogenase gene mutation. Conclusion: Our data illustrate that VASARI MRI features, especially intratumoral hemorrhage, contrast enhancement, and multicentricity, correlate strongly with glial tumor grade.

Objetivo: Determinar a correlação dos achados de imagem convencional e de difusão na ressonância magnética (RM) do encéfalo, com base nos critérios Visually AcceSAble Rembrandt Images (VASARI), com a classificação histopatológica de gliomas: gliomas de baixo grau e gliomas de alto grau. Materiais e Métodos: Imagens de RM pré-cirúrgicas de 178 pacientes com gliomas cerebrais e confirmação patológica foram avaliadas por dois neurorradiologistas quanto ao tamanho, localização e morfologia do tumor usando um padrão de imagem baseado nos critérios VASARI. Resultados: Na análise univariada, mais da metade das características avaliadas apresentou associação significativa com o grau do tumor. Hemorragia, restrição à difusão, invasão pial, realce e tumor sem realce cruzando a linha média foram as características com associação mais significativa. No modelo de regressão multivariada, a presença de realce e hemorragia manteve associação significativa com tumores de alto grau. A ausência de realce pelo meio de contraste e a restrição da difusão foram associados à presença da mutação do gene isocitrato desidrogenase. Conclusão: Nossos dados ilustram que as características de RM do VASARI, especialmente hemorragia intratumoral, presença de realce de contraste e multicentricidade, forneceram uma correlação importante com o grau da neoplasia glial.

Cerebral White Matter Alterations Associated With Oligodendrocyte Vulnerability in Organic Acidurias: Insights in Glutaric Aciduria Type I.

The white matter is an important constituent of the central nervous system, containing axons, oligodendrocytes, and its progenitor cells, astrocytes, and microglial cells. Oligodendrocytes are central for myelin synthesis, the insulating envelope that protects axons and allows normal neural conduction. Both, oligodendrocytes and myelin, are highly vulnerable to toxic factors in many neurodevelopmental and neurodegenerative disorders associated with disturbances of myelination. Here we review the main alterations in oligodendrocytes and myelin observed in some organic acidurias/acidemias, which correspond to inherited neurometabolic disorders biochemically characterized by accumulation of potentially neurotoxic organic acids and their derivatives. The yet incompletely understood mechanisms underlying the high vulnerability of OLs and/or myelin in glutaric acidemia type I, the most prototypical cerebral organic aciduria, are particularly discussed.

A rare cause of postpartum seizure: Cerebral tuberculoma.

Tuberculomas are rare and a life-threatening condition. Diagnosis followed by appropriate treatment can lead to complete resolution of the disease. A suggestive imaging study in an appropriate clinical setting can lead to the diagnosis. We describe a case of a postpartum woman with a headache and seizure in which eclampsia was the initial suspicion. Imaging exams demonstrated a solitary expansile lesion in the left parietal lobe suspicious of neoplasia. A biopsy, instead, confirmed a tuberculoma. In addition to eclampsia, many other differential diagnoses are possible in the context of seizures in pregnant and peripartum patients, including central nervous system tuberculosis. Brain imaging studies can be crucial in the diagnostic process.

Role of apparent diffusion map in the evaluation of retinoblastoma.

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.

Role of apparent diffusion map in the evaluation of retinoblastoma

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.

Evaluation of glial tumors: correlation between magnetic resonance imaging and histopathological analysis / Avaliação de tumores gliais por ressonância magnética e correlação com estudo histopatológico

Abstract Objective: To determine the correlation of conventional and diffusion-weighted imaging findings on magnetic resonance imaging (MRI) of the brain, based on Visually AcceSAble Rembrandt Images (VASARI) criteria, with the histopathological grading of gliomas: low-grade or high-grade. Materials and Methods: Preoperative MRI scans of 178 patients with brain gliomas and pathological confirmation were rated by two neuroradiologists for tumor size, location, and tumor morphology, using a standardized imaging feature set based on the VASARI criteria. Results: In the univariate analysis, more than half of the MRI characteristics evaluated showed a significant association with the tumor grade. The characteristics most significantly associated with the tumor grade were hemorrhage; restricted diffusion; pial invasion; enhancement; and a non-contrast-enhancing tumor crossing the midline. In a multivariable regression model, the presence of enhancement and hemorrhage maintained a significant association with high tumor grade. The absence of contrast enhancement and restricted diffusion were associated with the presence of an isocitrate dehydrogenase gene mutation. Conclusion: Our data illustrate that VASARI MRI features, especially intratumoral hemorrhage, contrast enhancement, and multicentricity, correlate strongly with glial tumor grade.

Resumo Objetivo: Determinar a correlação dos achados de imagem convencional e de difusão na ressonância magnética (RM) do encéfalo, com base nos critérios Visually AcceSAble Rembrandt Images (VASARI), com a classificação histopatológica de gliomas: gliomas de baixo grau e gliomas de alto grau. Materiais e Métodos: Imagens de RM pré-cirúrgicas de 178 pacientes com gliomas cerebrais e confirmação patológica foram avaliadas por dois neurorradiologistas quanto ao tamanho, localização e morfologia do tumor usando um padrão de imagem baseado nos critérios VASARI. Resultados: Na análise univariada, mais da metade das características avaliadas apresentou associação significativa com o grau do tumor. Hemorragia, restrição à difusão, invasão pial, realce e tumor sem realce cruzando a linha média foram as características com associação mais significativa. No modelo de regressão multivariada, a presença de realce e hemorragia manteve associação significativa com tumores de alto grau. A ausência de realce pelo meio de contraste e a restrição da difusão foram associados à presença da mutação do gene isocitrato desidrogenase. Conclusão: Nossos dados ilustram que as características de RM do VASARI, especialmente hemorragia intratumoral, presença de realce de contraste e multicentricidade, forneceram uma correlação importante com o grau da neoplasia glial.

The Role of MRI in Differentiating Demyelinating and Inflammatory (not Infectious) Myelopathies.

Demyelinating and inflammatory myelopathies represent a group of diseases with characteristic patterns in neuroimaging and several differential diagnoses. The main imaging patterns of demyelinating myelopathies (multiple sclerosis, neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, and myelin oligodendrocyte glycoprotein antibody-related disorder) and inflammatory myelopathies (systemic lupus erythematosus-myelitis, sarcoidosis-myelitis, Sjögren-myelitis, and Behçet's-myelitis) will be discussed in this article, highlighting key points to the differential diagnosis.

The longitudinal progression of MRI changes in pre-ataxic carriers of SCA3/MJD.

BACKGROUND: The natural history of magnetic resonance imaging (MRI) in pre-ataxic stages of spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD) is not well known. We report cross-sectional and longitudinal data obtained at this stage. METHODS: Baseline (follow-up) observations included 32 (17) pre-ataxic carriers (SARA < 3) and 20 (12) related controls. The mutation length was used to estimate the time to onset (TimeTo) of gait ataxia. Clinical scales and MRIs were performed at baseline and after a median (IQR) of 30 (7) months. Cerebellar volumetries (ACAPULCO), deep gray-matter (T1-Multiatlas), cortical thickness (FreeSurfer), cervical spinal cord area (SCT) and white matter (DTI-Multiatlas) were assessed. Baseline differences between groups were described; variables that presented a p < 0.1 after Bonferroni correction were assessed longitudinally, using TimeTo and study time. For TimeTo strategy, corrections for age, sex and intracranial volume were done with Z-score progression. A significance level of 5% was adopted. RESULTS: SCT at C1 level distinguished pre-ataxic carriers from controls. DTI measures of the right inferior cerebellar peduncle (ICP), bilateral middle cerebellar peduncles (MCP) and bilateral medial lemniscus (ML), also distinguished pre-ataxic carriers from controls, and progressed over TimeTo, with effect sizes varying from 0.11 to 0.20, larger than those of the clinical scales. No MRI variable showed progression over study time. DISCUSSION: DTI parameters of the right ICP, left MCP and right ML were the best biomarkers for the pre-ataxic stage of SCA3/MJD. TimeTo is an interesting timescale, since it captured the longitudinal worsening of these structures.

Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings.

Hypertrophic pachymeningitis (HP) is a rare and chronic inflammatory disorder presenting as localized or diffuse thickening of the dura mater. It can be idiopathic or an unusual manifestation of immune-mediated, infectious, and neoplastic conditions. Although some cases may remain asymptomatic, HP can lead to progressive headaches, cranial nerve palsies, hydrocephalus, and other neurological complications, which makes its recognition a fundamental step for prompt treatment. Regarding the diagnosis workup, enhanced MRI is the most useful imaging method to evaluate dural thickening. This article addresses the MR imaging patterns of immune-mediated HP, including immunoglobulin G4-related disease, neurosarcoidosis, granulomatosis with polyangiitis, rheumatoid pachymeningitis, and idiopathic HP. The main infectious and neoplastic mimicking entities are also discussed with reference to conventional and advanced MR sequences.

Primary somatosensory cortex and periaqueductal gray functional connectivity as a marker of the dysfunction of the descending pain modulatory system in fibromyalgia.

Background: Resting-state functional connectivity (rs-FC) may aid in understanding the link between pain-modulating brain regions and the descending pain modulatory system (DPMS) in fibromyalgia (FM). This study investigated whether the differences in rs-FC of the primary somatosensory cortex in responders and non-responders to the conditioned pain modulation test (CPM-test) are related to pain, sleep quality, central sensitization, and the impact of FM on quality of life. Methods: This cross-sectional study included 33 females with FM. rs-FC was assessed by functional magnetic resonance imaging. Change in the numerical pain scale during the CPM-test assessed the DPMS function. Subjects were classified either as non-responders (i.e., DPMS dysfunction, n = 13) or responders (n = 20) to CPM-test. A generalized linear model (GLM) and a receiver operating characteristic (ROC) curve analysis were performed to check the accuracy of the rs-FC to differentiate each group. Results: Non-responders showed a decreased rs-FC between the left somatosensory cortex (S1) and the periaqueductal gray (PAG) (P < 0.001). The GLM analysis revealed that the S1-PAG rs-FC in the left-brain hemisphere was positively correlated with a central sensitization symptom and negatively correlated with sleep quality and pain scores. ROC curve analysis showed that left S1-PAG rs-FC offers a sensitivity and specificity of 85% or higher (area under the curve, 0.78, 95% confidence interval, 0.63-0.94) to discriminate who does/does not respond to the CPM-test. Conclusions: These results support using the rs-FC patterns in the left S1-PAG as a marker for predicting CPM-test response, which may aid in treatment individualization in FM patients.

Primary central nervous system lymphoma in a patient with multiple sclerosis using fingolimod.

Primary central nervous system lymphoma (PCNSL) is an uncommon lymphoproliferative disease associated with immunosuppression. Here, we report the case of a patient with multiple sclerosis, under treatment with fingolimod (FTY720, Gilenya) for 4 years, who developed this condition. Although the causal relationship cannot be established, there are cases in the literature that describe the appearance of lymphoma after the use of this medication. Considering the high mortality of PCNSL, epidemiological studies are necessary to establish a relationship between its arising and the use of immunosuppressants.

Imaging manifestations of von Hippel-Lindau disease: an illustrated guide focusing on abdominal manifestations.

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.

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.

Idiopathic intracranial hypertension: an illustrated guide for the trainee radiologist.

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.

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.

Imaging manifestations of von Hippel-Lindau disease: an illustrated guide focusing on abdominal manifestations / Manifestações de imagem da doença de von Hippel-Lindau: um guia ilustrado das manifestações abdominais

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 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.

Idiopathic intracranial hypertension: an illustrated guide for the trainee radiologist / Hipertensão intracraniana idiopática: um guia ilustrado para o residente de radiologia

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 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.

Common and uncommon neuroimaging manifestations of ataxia: an illustrated guide for the trainee radiologist. Part 1 - acquired diseases.

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.

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.

Common and uncommon neuroimaging manifestations of ataxia: an illustrated guide for the trainee radiologist. Part 2 - neoplastic, congenital, degenerative, and hereditary diseases.

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.

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.

Common and uncommon neuroimaging manifestations of ataxia: an illustrated guide for the trainee radiologist. Part 2 - neoplastic, congenital, degenerative, and hereditary diseases / Manifestações de neuroimagem comuns e incomuns na ataxia: um guia ilustrado para radiologistas em treinamento. Parte 2 - doenças neoplásicas, congênitas, degenerativas e hereditárias

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, 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.

Common and uncommon neuroimaging manifestations of ataxia: an illustrated guide for the trainee radiologist. Part 1 - acquired diseases / Manifestações de neuroimagem comuns e incomuns na ataxia: um guia ilustrado para radiologistas em treinamento. Parte 1 - doenças adquiridas

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.

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.

Imaging manifestations of von Hippel-Lindau disease: an illustrated guide focusing on the central nervous system.

Von Hippel-Lindau (VHL) disease is a rare, autosomal dominant inherited syndrome that affects the germline of the VHL gene, a tumor suppressor gene. VHL disease is characterized by the multisystemic development of a variety of benign and malignant tumors, especially in the central nervous system (CNS). Such tumors include retinal and CNS hemangioblastomas, as well as endolymphatic sac tumors. The various tumor sites are responsible for the diversity of signs and symptoms related to the disease. The mean age at symptom onset is 33 years. Despite medical advances, the average life expectancy of patients with VHL disease is 49 years. Imaging plays a pivotal role in the clinical diagnosis and is essential to the follow-up of patients with VHL disease. This pictorial essay describes characteristic CNS manifestations of VHL disease-related tumors that all radiology residents should be aware of.

A doença de von Hippel-Lindau (VHL) é uma síndrome hereditária autossômica dominante rara que afeta a linha germinativa do gene VHL, um gene supressor tumoral. A doença de VHL é caracterizada pelo desenvolvimento multissistêmico de uma variedade de tumores benignos e malignos, especialmente no sistema nervoso central (SNC). Dentre eles, destacam-se hemangioblastomas retinianos e do SNC, e o tumor do saco endolinfático. Os diferentes locais dos tumores justificam a diversidade de sinais e sintomas relacionados à doença, que usualmente se manifestam com a idade média de 33 anos. Apesar dos avanços da medicina, a expectativa de vida média desses pacientes é de 49 anos. Exames de imagem têm papel fundamental no diagnóstico e são essenciais no seguimento dos pacientes com doença de VHL. Este ensaio iconográfico descreve as manifestações características dos tumores do SNC relacionados à doença de VHL que todos os residentes de radiologia devem saber.