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A number of conditions can mimic amyotrophic lateral sclerosis (ALS), which are in general excluded by neurophysiological and neuroimaging investigation. We present a novel mimicking disorder. A 58-year-old male, without relevant past medical history, presented with a 7-year history of progressive paraparesis. On examination, he had bilateral thigh atrophy, fasciculations, and asymmetric paraparesis (severe on the left side). Upper motor neuron signs were present in the lower limbs, with normal sensory examination. Needle EMG disclosed mild chronic neurogenic changes in the lower limbs. Brain and spinal cord neuroimaging was normal, namely, in the dorso-lumbar segment. Lumbar puncture showed mild hyperproteinorachia. Diagnosis of slowly progressive (possible) ALS was established. One year later, he required a bilateral support to walk, and neurological examination revealed weak tendon reflexes, abnormal pinprick, and proprioceptive sensation in the legs. Repeated lumbar MRI showed an extensive spinal cord oedema from T7 to the conus with multiple perimedullary vessel flow voids suggestive of a vascular malformation. Conventional angiography revealed a spinal dural arteriovenous fistula in L2–L3 with the left L4 lumbar branch as the afferent artery. Dural arteriovenous fistula is the most common vascular malformation of the spinal cord, despite being rare. It leads to arterialization of spinal veins, causing venous hypertension, spinal cord oedema, and ischaemia. The clinical picture includes a stepwise, sometimes fluctuant, myeloradiculopathy. In this case, EMG changes did not meet Awaji criteria. This case reinforces the need to critically follow atypical cases to ascertain clinical progression in patients with suspected ALS.

Abstract Background Parkinsonian variant of multiple system atrophy is a neurodegenerative disorder frequently misdiagnosed as Parkinson’s disease. No early imaging biomarkers currently differentiate these disorders. Methods Simple visual imaging analysis of the substantia nigra and locus coeruleus in neuromelanin-sensitive magnetic resonance imaging and nigrosome 1 in susceptibility-weighted sequences was performed in thirty patients with parkinsonian variant of multiple system atrophy fulfilling possible/probable second consensus diagnostic criteria. The neuromelanin visual pattern was compared to patients with Parkinson’s disease with the same disease duration (n = 10) and healthy controls (n = 10). Substantia nigra semi-automated neuromelanin area/signal intensity was compared to the visual data. Results Groups were similar in age, sex, disease duration, and levodopa equivalent dose. Hoehn & Yahr stage was higher in parkinsonian multiple system atrophy patients, 69% of whom had normal neuromelanin size/signal, significantly different from Parkinson’s disease patients, and similar to controls. Nigrosome 1 signal was lost in 74% of parkinsonian multiple system atrophy patients. Semi-automated neuromelanin substantia nigra signal, but not area, measurements were able to differentiate groups. Conclusions In patients with parkinsonism, simple visual magnetic resonance imaging analysis showing normal neuromelanin substantia nigra and locus coeruleus, combined with nigrosome 1 loss, allowed the distinction of the parkinsonian variant of multiple system atrophy from Parkinson’s disease and healthy controls. This easy and widely available method was superior to semi-automated measurements in identifying specific imaging changes in substantia nigra and locus coeruleus.

Background: Previous studies suggested that Parkinson's Disease (PD) patients could have an increased risk of atrial fibrillation. However, data supporting this association is not robust. We aimed to compare the potential risk of atrial fibrillation associated with PD in an age and gender matched case-control study, comparing the p-wave indexes from electrocardiograms and clinical risk scores among groups.Methods: A cross-sectional case-control study was performed. All subjects included in the analysis were clinically evaluated and subjected to a 12-lead electrocardiogram. Two blinded independent raters measured the p-wave duration. Subjects were classified as having normal P-wave duration (

Introduction: There is limited evidence regarding long-term outcomes of aneurysmal subarachnoid hemorrhage survivors. Most follow-up programs are relatively short and focused on physical functions. Endovascular aneurysmal embolization enables recovery of normal vascular architecture. However, there is growing evidence that neuropsychological and behavior sequelae can significantly impact the lives of these patients, even when treatment is successful. In this study, we reviewed cognition, psychiatric and neuropsychological symptoms, global functionality, and health-related quality of life 10 to 12 years after an aneurysmal subarachnoid hemorrhage. Material and Methods: A cross-sectional observational study was carried out in a university hospital. All cases of aneurysmal subarachnoid hemorrhage admitted between January 2004 and December 2006 and endovascularly treated were reviewed. Participants underwent a neuropsychological evaluation and a clinical interview with a psychiatrist. Results: Fourteen patients participated in the study. Almost 70% (n = 10) showed cognitive impairment; in more than 40% (n = 6) of the subjects, significant symptoms of anxiety were identified, and 35% (n = 5) were classified as having clinical depression. Relevant posttraumatic symptoms were reported by more than 70% (n = 10) of patients, and almost 30% (n = 4) showed other moderate neuropsychiatric symptoms. Overall, health-related quality of life was impaired, and personality changes were frequently reported by the participants and their relatives. Discussion: A significant prevalence of ongoing deficits in high-level functioning and reduced health-related quality of life were observed in a sample of young and professionally active individuals that were successfully treated and discharged from follow-up consultations. Conclusion: There is a need for better follow-up strategies, targeting more subtle deficits and psychological symptoms after aneurysmal subarachnoid hemorrhage.

Disseminated necrotizing leukoencephalopathy (DNL) is characterized by multiple microscopic foci of white matter necrosis. DNL was initially thought to be exclusively associated with immunosuppression conditions but it has been recently described in immunocompetent patients in septic shock. A 90-year-old immunocompetent woman with no previous neurological impairment presented with septic shock and drowsiness that responded well to therapy with clinical improvement and a full neurological recovery. Unexpectedly deterioration with progression to coma occurred. Investigation excluded other causes and Magnetic Resonance Imaging (MRI) was consistent with the diagnosis of DNL showing bilateral multifocal white matter lesions with a nonvascular pattern with restricted diffusion. Neurological impairment persisted with progression to death. DNL is an unexpected diagnosis in an immunocompetent patient. We compared the present case to those found in the literature of DNL complicating septic shock and discuss the antemortem diagnosis based on MRI findings.

Introduction: Cognitive and behavioral impairments are considered to occur frequently in amyotrophic lateral sclerosis/motor neuron disease (MND). Rarely, apraxia has been reported in MND. Orofacial, or buccofacial, apraxia is characterized by a loss of voluntary control of facial, lingual, pharyngeal and masticatory muscles in the presence of preserved reflexive and automatic functions of the same muscles. Methods: We report a patient with MND who presented with spastic dysarthria and asymmetric orofacial apraxia. She progressed to frontotemporal dementia (FTD). Results: Clinical and neurophysiological examinations were suggestive of bulbar-onset MND-FTD. Tractography showed a reduction of fractional anisotropy in the centrum semiovale, corona radiata, corticomedullary pathway and inferior aspect of the medulla; the changes were more severe on the left side. To our knowledge, this is the first report of an asymmetric presentation of an apraxic syndrome in MND-FTD.

Introdução / Objectivos: A angiografia cerebral é uma técnica efectuada para a detecção de anomalias dos vasos sanguíneos cerebrais. Para a sua realização é fundamental que o neurorradiologista conheça o padrão de desenvolvimento anatómico que ocorre durante a morfogénese cardiovascular e as respectivas anomalias que podem condicionar a sua realização. Métodos: A propósito de um achado acidental de um duplo arco aórtico aquando da realização de uma angiografia os autores procederam a uma revisão bibliográfica dos aspectos característicos da morfogénese cardiovascular e das principais anomalias que podem ocorrer durante este processo. Discussão: A existência de um duplo arco aórtico constitui o padrão embrionário presente às seis semanas de gestação que resulta do processo de progressão/regressão dos seis pares de arcos faríngeos arteriais; habitualmente regride à sétima semana de gestação com a normal progressão da morfogénese cardiovascular. Caso o quarto arco faríngeo direito e a aorta dorsal direita não regridam, este padrão de duplo arco aórtico mantém-se mesmo após o nascimento. Existem vários sintomas associados a esta anomalia do desenvolvimento mas este padrão pode estar presente num adulto assintomático que, tal como no caso apresentado, é identificado acidentalmente. Conclusão: O conhecimento por parte do neurorradiologista da morfogénese cardiovascular e suas principais anomalias conduz a uma optimização da técnica de angiografia cerebral.

Introdução: A leucoencefalopatia mutifocal progressiva (LEMP) é uma doença desmielinizante subaguda do sistema nervoso central causada pelo vírus neurotrópico John Cunningham (JC), que ocorre em doentes com imunossupressão grave, nomeadamente em doentes infectados pelo vírus da imunodeficiência humana (VIH). As lesões de LEMP do parênquima encefálico têm uma natureza progressiva, sendo importante a sua caracterização e estudo evolutivo, nomeadamente a avaliação da resposta terapêutica. Material e Métodos: Os autores procederam à revisão dos aspectos imagiológicos de estudos de Ressonância Magnética com imagens ponderadas em difusão em lesões de LEMP do parênquima encefálico em quatro doentes, caracterizando os diferentes componentes lesionais nas imagens de ressonância magnética (RM) ponderada em difusão (DWI e ADC) e correlacionando-os com os dados previamente publicados na literatura, nomeadamente quanto à correlação histológica e evolução temporal das lesões. Resultados: Os quatro exames avaliados demonstraram uma heterogeneidade lesional no estudo de RM ponderada em difusão, com restrição à difusão na periferia de cada lesão. Discussão/Conclusão: O estudo de RM com imagens ponderadas em difusão permite caracterizar as lesões de LEMP do parênquima encefálico, evidenciando a sua heterogeneidade e assincronia, dados importantes no conhecimento da evolução destas lesões.

Introdução / Objectivos: A tomografia computorizada (TC) da coluna vertebral é dos exames neurorradiológicos mais frequentemente realizados, com grande número de indicações, em particular para estudo de patologia degenerativa. Diversos sinais imagiológicos de caracterização de alterações nos estudos imagiológicos da coluna lombar foram descritos, inicialmente na radiologia convencional, mas com aplicação e valor diagnóstico nos estudos por TC, sendo objectivo deste trabalho a sua identificação e integração. Métodos: Os autores efectuaram uma revisão dos principais sinais imagiológicos de estudo da coluna vertebral descritos na literatura. Resultados e Discussão: Descreveram-se esses sinais imagiológicos na coluna vertebral, correlacionando-os com o diagnóstico das condições clínicas a eles associadas. Conclusão: O conhecimento destes sinais, inicialmente descritos na radiologia convencional, reveste-se de particular importância para o neurorradiologista, dado poderem constituir pistas importantes para o diagnóstico de uma grande variedade de situações clínicas.

Acute confusion and memory loss associated with asymmetrical mesiotemporal hyperintensity on T2-weighted MRI are characteristic of herpes encephalitis. The authors report the case of a patient with these symptoms and MRI presentation who had neurosyphilis. Recently clinical and imaging patterns usually associated with herpes simplex encephalitis have been seen in patients with neurosyphilis. Because syphilis is “The Great Pretender” not only clinically but also in imaging and because its numbers are rising, it must be sought as a differential diagnosis.

Medulloblastoma is a brain tumor of neuroepithelial origin, frequent in children but rare in adults. The imaging pattern is well studied in the pediatric group thought there is controversy about the imaging characteristics in adults. We report CT and MRI imaging findings of 8 adult patients with cerebellar medulloblastoma. The mean age was 29.6 years (16-65 years). The male: female rate was 5:3. Most were lateral, located in the cerebellar hemisphere (63%). They were hyperdense on CT (67%). On the MRI they were all hypointense on T1, hyperintense on T2, with gadolinium enhancement of variable pattern. In 7 cases there were cysts/intratumoral necrosis. It was seen calcifications in 2 cases. Four patients presented hydrocephalus. In 2 cases there was no perilesional edema. All had well defined margins and superficial extension. Dural involvement was seen in 7, one of which with lateral venous sinus compromise, and brainstem invasion was seen in 1 case. The imaging findings of medulloblastomas in adults are unspecific and different from those in child. They should be considered in the differential diagnosis of cerebellar tumor in adults, especially if they are hyperdense on CT, with well defined margins, with superficial extension and with dural involvement.

Multisection computed tomography (MSCT) was introduced in 1998 and improved neuroimaging quality, in particular concerning acute stroke. Previously, non-enhanced CT was used not only to detect early stroke signs, but also to exclude hemorrhage and non-vascular pathology responsible for the acute neurological deficit. Nowadays, using Perfusion CT (PCT) it is possible to obtain a functional study of the cerebral hemodinamics after injection of a fast bolus of contrast. Multi-voxel analysis of the time-attenuation curves delivers colour maps of Cerebral Blood Flow (CBF), Mean Time Transit (MTT) and Cerebral Blood Flow (CBF). Based on specific patterns of hemodinamic changes it is possible to differentiate between irreversible and reversible brain damage--"tissue at risk", which is essential for choosing an appropriate therapy. The authors will discuss data acquisition, post-processing and image interpretation and analysis starting from two clinical examples.

Neuroepithelial cysts, heterogeneous group of lesions, are fluid collections similar to CSF, lined by epithelial cells, frequently found in the lateral ventricles and choroidal fissure; intraparenchymal cysts are relatively uncommon. We report CT and MRI imaging findings of four intraparenchymal neuroepithelial cysts, two of which have histological confirmation of the diagnosis.

Introdução – A cintigrafia de perfusão do miocárdio (CPM) desempenha um importante papel no diagnóstico, avaliação e seguimento de pacientes com doença arterial coronária, sendo o seu processamento realizado maioritariamente de forma semiautomática. Uma vez que o desempenho dos técnicos de medicina nuclear (TMN) pode ser afetado por fatores individuais e ambientais, diferentes profissionais que processem os mesmos dados poderão obter diferentes estimativas dos parâmetros quantitativos (PQ). Objetivo – Avaliar a influência da experiência profissional e da função visual no processamento semiautomático da CPM. Analisar a variabilidade intra e interoperador na determinação dos PQ funcionais e de perfusão. Metodologia – Selecionou-se uma amostra de 20 TMN divididos em dois grupos, de acordo com a sua experiência no software Quantitative Gated SPECTTM: Grupo A (GA) – TMN ≥600h de experiência e Grupo B (GB) – TMN sem experiência. Submeteram-se os TMN a uma avaliação ortóptica e ao processamento de 21 CPM, cinco vezes, não consecutivas. Considerou-se uma visão alterada quando pelo menos um parâmetro da função visual se encontrava anormal. Para avaliar a repetibilidade e a reprodutibilidade recorreu-se à determinação dos coeficientes de variação, %. Na comparação dos PQ entre operadores, e para a análise do desempenho entre o GA e GB, aplicou-se o Teste de Friedman e de Wilcoxon, respetivamente, considerando o processamento das mesmas CPM. Para a comparação de TMN com visão normal e alterada na determinação dos PQ utilizou-se o Teste Mann-Whitney e para avaliar a influência da visão para cada PQ recorreu-se ao coeficiente de associação ETA. Diferenças estatisticamente significativas foram assumidas ao nível de significância de 5%. Resultados e Discussão – Verificou-se uma reduzida variabilidade intra (

Introduction – Myocardial perfusion scan (MPS) has an important role in the diagnosis, evaluation and follow-up of patients with coronary artery disease being the semiautomatic processing widely used for routine clinical practice. Since the nuclear medicine technologists (NMT) performance might be affected by individual and environmental factors, different professionals that process the same data will provide different estimations of quantitative parameters (QP). Aim – Evaluate the influence of NMT professional experience and visual function on the MPS semiautomatic processing. Analysis of the intra and inter variability considering the function and perfusion QP measured. Methodology – Twenty subjects were selected and categorized in two groups according to their experience on the Quantitative Gated SPECTTM software: Group A (GA) – NMT ≥600h and Group B (GB) – NMT without experience. All NMT underwent an orthoptic evaluation and processed 21 MPS five times in a non-consecutive mode. It was assumed to be an altered vision when at least one visual function parameter was found abnormal. Coefficient of variation, %, was calculated to evaluate repeatability and reproducibility. Friedman’s and Wilcoxon tests were applied to compare QP between operators and to analyze GA and GB performance, respectively, considering the processing of the same MPS. To compare the NMT with normal and altered vision it was used the Mann-Whitney Test, and to evaluate the visual function influence in each QP was performed the ETA association coefficient. It was assumed statistically significant differences at a significance level of 5%. Results and Discussion – Low intra (