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Background and Purpose The cardiovascular risk in Parkinson’s disease (PD) remains uncertain and controversial. Some studies suggest PD patients present an increased risk of cerebrovascular disease. We aimed to study the prevalence of neuroimaging cerebrovascular biomarkers in PD patients compared to controls, using an accurate and complete magnetic resonance (MR) imaging evaluation. Material and Methods Neuroimaging sub-study within a larger cross-sectional case–control study. An enriched subgroup of PD patients (≤10 years since diagnosis) with at least a moderate cardiovascular mortality risk based on a Systematic COronary Risk Evaluation (SCORE) was compared to community-based controls regarding neuroimaging biomarkers. Patients underwent a high-resolution T1-weighted MR imaging sequence at 3.0 T to visualize neuromelanin. A 3D SWI FFE, sagittal 3D T1-weighted, axial FLAIR and diffusion-weighted image sequences were obtained. Results The study included 47 patients, 24 with PD and 23 controls. PD patients presented a reduced area and signal intensity of the substantia nigra and locus coeruleus on neuromelanin-sensitive MR. The median SCORE was 5% in both groups. No significant differences regarding white matter hyperintensities (OR 4.84, 95% CI 0.50, 47.06), lacunes (OR 0.43, 95% CI 0.07, 2.63), microbleeds (OR 0.64, 95% CI 0.13, 3.26), or infarcts (0.95, 95% CI 0.12, 7.41) was found. The frequency of these neuroimaging biomarkers was very low in both groups. Conclusion The present study does not support an increased prevalence of neuroimaging cerebrovascular biomarkers in PD patients.

© The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attri- bution 4.0 International License, which permits use, sharing, adapta- tion, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/., Purpose: There is limited data concerning neuroimaging findings and longitudinal evaluation of familial cerebral cavernous malformations (FCCM) in children. Our aim was to study the natural history of pediatric FCCM, with an emphasis on symptomatic hemorrhagic events and associated clinical and imaging risk factors. Methods: We retrospectively reviewed all children diagnosed with FCCM in four tertiary pediatric hospitals between January 2010 and March 2022. Subjects with first available brain MRI and [Formula: see text] 3 months of clinical follow-up were included. Neuroimaging studies were reviewed, and clinical data collected. Annual symptomatic hemorrhage risk rates and cumulative risks were calculated using survival analysis and predictors of symptomatic hemorrhagic identified using regression analysis. Results: Forty-one children (53.7% males) were included, of whom 15 (36.3%) presenting with symptomatic hemorrhage. Seven symptomatic hemorrhages occurred during 140.5 person-years of follow-up, yielding a 5-year annual hemorrhage rate of 5.0% per person-year. The 1-, 2-, and 5-year cumulative risks of symptomatic hemorrhage were 7.3%, 14.6%, and 17.1%, respectively. The latter was higher in children with prior symptomatic hemorrhage (33.3%), CCM2 genotype (33.3%), and positive family history (20.7%). Number of brainstem (adjusted hazard ratio [HR] = 1.37, P = 0.005) and posterior fossa (adjusted HR = 1.64, P = 0.004) CCM at first brain MRI were significant independent predictors of prospective symptomatic hemorrhage. Conclusion: The 5-year annual and cumulative symptomatic hemorrhagic risk in our pediatric FCCM cohort equals the overall risk described in children and adults with all types of CCM. Imaging features at first brain MRI may help to predict potential symptomatic hemorrhage at 5-year follow-up.

Imaging studies are crucial adjuncts when studying acute and chronic diseases, so pregnant and lactating women are as likely to be evaluated with one of the available imaging modalities. Due to the specific condition of the mother and child in this time period it is crucial to make an appropriate selection of imaging studies. We review the existing literature and analyse the latest evidence and guidelines regarding neuroimaging safety during pregnancy and lactation, proposing an algorithm of action based on risk/benefits assessment. Choosing the most appropriate neuroimaging modality implicates assessing the pretest pertinence of the study—the possibility of a serious treatable neurologic disease, pondering what is the most useful imaging modality for the diagnosis and evaluating the associated risks. Among physicians (and patients), however, the risk component is perhaps the least well understood, with misperceptions regarding safety and potential hazards. Computed tomography (CT) risks are principally related to ionizing radiation and intravenous (IV) administration of iodinated contrast. However, as very low risks for the mother and foetus have been reported and CT remains the most available tool for initial rapid diagnosis of acute neurological conditions, it should not be withheld in urgent situations. Magnetic resonance imaging (MRI), unlike CT, does not use ionizing radiation or iodinated contrast mediums, having the best anatomical detail possible. However, there are some usage safety concerns regarding the magnetic field strength and gadolinium-based contrast use. There are lacking longitudinal and prospective studies to sustain evidence based choices of imaging studies during pregnancy and lactation. Ultimately the decision should be based on the risk/benefit, taking into account the patient’s safety, care and outcomes. However, using a specific algorithm can guide decisions in daily clinical practice.

© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2022, Purpose: The aim of the study was to assess the prevalence and characteristics of spinal cord cavernous malformations (SCCM) and intraosseous spinal vascular malformations (ISVM) in a pediatric familial cerebral cavernous malformation (FCCM) cohort and evaluate clinico-radiological differences between children with (SCCM +) and without (SCCM-) SCCM. Methods: All patients with a pediatric diagnosis of FCCM evaluated at three tertiary pediatric hospitals between January 2010 and August 2021 with [Formula: see text] 1 whole spine MR available were included. Brain and spine MR studies were retrospectively evaluated, and clinical and genetic data collected. Comparisons between SCCM + and SCCM- groups were performed using student-t/Mann-Whitney or Fisher exact tests, as appropriate. Results: Thirty-one children (55% boys) were included. Baseline spine MR was performed (mean age = 9.7 years) following clinical manifestations in one subject (3%) and as a screening strategy in the remainder. Six SCCM were detected in five patients (16%), in the cervico-medullary junction (n = 1), cervical (n = 3), and high thoracic (n = 2) regions, with one appearing during follow-up. A tendency towards an older age at first spine MR (P = 0.14) and [Formula: see text] 1 posterior fossa lesion (P = 0.13) was observed in SCCM + patients, lacking statistical significance. No subject demonstrated ISVM. Conclusion: Although rarely symptomatic, SCCM can be detected in up to 16% of pediatric FCCM patients using diverse spine MR protocols and may appear de novo. ISVM were instead absent in our cohort. Given the relative commonality of asymptomatic SCCM, serial screening spine MR should be considered in FCCM starting in childhood.

© 2020 International Parkinson and Movement Disorder Society, Despite well-established Parkinson’s disease (PD) diagnostic criteria, diagnosis can still be challenging, with a high rate of misdiagnosis in atypical cases, especially in early disease stages. The majority of misdiagnoses are related to the differential diagnosis of PD with atypical parkinsonian disorders (APD), such as multiple system atrophy–parkinsonian type (MSA-P), progressive supranuclear palsy (PSP), or corticobasal degeneration, as well as essential tremor (ET) and other forms of parkinsonism (drug-induced, vascular, functional).

Background Nigrostriatal dopaminergic neuron degeneration with loss of Substantia Nigra (SN) and Locus Coeruleus (LC) neurons, as well as disruption of iron homeostasis, have been reported in Huntington’s Disease (HD), but literature is still scarce. Recently developed magnetic resonance imaging (MRI) biomarkers, such as neuromelanin-sensitive MRI (NM-MRI) and Nigrosome-1 (N1) visualization on susceptibility-weighted imaging (SWI), permit in vivo evaluation of changes in NM containing neurons of the SN and LC and in iron deposition. Aim The aim of this study was to use these novel MRI methods to further clarify SN and LC involvement in HD, through visual analysis. Methods Cross-sectional comparative study analyzing NM and N1 in HD patients and healthy controls (HC). Visual analysis of the SN and LC NM and of the N1 was performed. Fisher’s exact test was used to compare visual analysis ratings between groups. Additionally, SN NM area was obtained using semi-automatic segmentation and median area was used for non-parametric analysis. Results Twelve HD patients and 13 HC were included. LC and SN NM signal intensity were found to be consistently reduced in HD. Visual and semi-quantitative analysis of the SN-NM area did not show significant differences between the groups. No significant differences were found in the analysis of the N1. Conclusions Reduction of LC and SN NM signal on NM-MRI corroborates previous neuropathological findings suggesting striatonigral dysfunction in symptomatic HD patients. Our results suggest that visual analysis may be useful for the evaluation of NM-MRI and N1 in HD patients in clinical settings.

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.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.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.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.There is a need for better follow-up strategies, targeting more subtle deficits and psychological symptoms after aneurysmal subarachnoid hemorrhage.Introdução: As evidências sobre a evolução a longo prazo dos sobreviventes de uma hemorragia subaracnoideia aneurismática são relativamente limitadas. A maioria dos programas de follow-up têm uma curta duração e são focados principalmente nas funções motoras. Apesar da embolização aneurismática endovascular permitir uma recuperação da arquitetura vascular normal, há evidências crescentes de que certas sequelas neuropsicológicas e comportamentais podem afetar significativamente a vida desses pacientes, a longo prazo, mesmo quando o tratamento é bem-sucedido. Neste estudo, analisamos os sintomas cognitivos, psiquiátricos e neuropsicológicos, a funcionalidade global e a qualidade de vida relacionada com a saúde, 10 a 12 anos após uma hemorragia subaracnoideia aneurismática. Material e Métodos: Um estudo observacional, transversal, foi realizado num hospital universitário. Todos os casos de hemorragia subaracnoideia aneurismática, admitidos entre janeiro de 2004 e dezembro de 2006, tratados endovascularmente, foram revistos. Os participantes foram sujeitos a uma avaliação neuropsicológicas e a uma entrevista clínica com um psiquiatra. Resultados: Participaram no estudo 14 doentes. Cerca de 70% (n = 10) apresentavam compromisso cognitivo; em mais de 40% (n = 6) foram identificados sintomas significativos de ansiedade e 35% (n = 5) foram classificados como tendo depressão clínica. Sintomas de stress pós-traumático relevantes foram relatados por mais de 70% (n = 10) e quase 30% (n = 4) apresentavam sintomas neuropsiquiátricos moderados. Em geral, a qualidade de vida relacionada com o estado de saúde encontrava-se reduzida e relatos de alterações de personalidade foram frequentemente feitos pelos participantes e seus familiares. Discussão: Uma prevalência significativa de défices em altos níveis de funcionamento e uma redução da qualidade de vida relacionada com a saúde foi observada numa amostra de indivíduos jovens e profissionalmente ativos, que foram tratados com sucesso e tiveram alta das consultas de seguimento. Conclusão: São necessárias melhores estratégias de follow-up, visando défices cognitivos e sintomas psicológicos mais subtis, após uma hemorragia subaracnoideia aneurismática.

© 2021 European Academy of Neurology, Background: The relationship between Parkinson's disease (PD) and cardiovascular and cerebrovascular disease is not yet well established. Recent data suggest an increased risk of myocardial infarction and stroke in PD patients. Therefore, we designed a study to assess surrogate markers of cardiovascular and cerebrovascular risk in PD. Methods: We conducted a case-control study comparing PD patients recruited from a Movement Disorders Unit with controls randomly invited from a primary healthcare center. All participants underwent a detailed clinical evaluation, including medical history, physical assessment, carotid ultrasound, blood and urine analysis, and 24-h ambulatory blood pressure monitoring. The primary outcome was the carotid intima-media thickness (CIMT). Results: We included 102 participants in each study arm. No significant difference was found in the CIMT among groups (MD: 0.01, 95% CI: -0.02, 0.04). Carotid plaques were more frequent in PD patients (OR: 1.90, 95% CI: 1.02, 3.55), although the lipid profile was more favorable in this group (LDL MD: -18.75; 95% CI: -10.69, -26.81). Nocturnal systolic blood pressure was significantly higher in PD patients (MD: 4.37, 95% CI: 0.27, 8.47) and more than half of the PD patients were non-dippers or reverse dippers (OR: 1.83, 95% CI: 1.04, 3.20). Conclusion: We did not find a difference in CIMT between PD and controls. A higher frequency of carotid plaques and abnormal dipper profile supports the hypothesis that PD patients are not protected from cardiovascular and cerebrovascular disease., M.A. was awarded a scholarship from Fundação para a Ciência e Tecnologia (SFRH/BD/129700/2017). IMM - UID/BIM/50005/2019, project funded by Fundação para a Ciência e a Tecnologia (FCT)/Ministério da Ciência, Tecnologia e Ensino Superior (MCTES) through Fundos do Orçamento de Estado

© 2020 International Parkinson and Movement Disorder Society., We read with interest the study by Choe and colleagues who increases emphasis on the heart–brain axis in Parkinson’s disease (PD). They have studied the relation between subclinical cardiac markers and motor impairment (Unifed Parkinson’s Disease Rating Scale Part III and Hoehn and Yahr stage) and cognition (Montreal Cognitive Assessment[MoCA]) concluding that patients with PD presented higher levels of N-terminal pro-B-type natriuretic peptide(NT-proBNP) compared with healthy controls matched forage, sex, and cardiovascular risk factors; increased levels of NT-proBNP and high-sensitivity troponin I were associated with worse motor impairment (Unifed Parkinson’s DiseaseRating Scale Part III and Hoehn and Yahr stage); and MoCA was inversely associated to high-sensitivity tropo-nin I and NT-proBNP levels only in unadjusted models among patients with PD.The population included were patients with advanced-stage PD (12 years of disease durationin average).

Radiation recall myelitis is a rare and unpredictable inflammatory reaction that occurs in previously irradiated areas, triggered by a chemotherapy agent. The diagnosis is difficult due to the possibility of a long time-lapse between radiotherapy (RT) and the occurrence of usually unspecific symptoms. We report the case of a 48-year-old woman with stage IV metastatic breast cancer who presented with progressive numbness and weakness in the lower limbs arising 4 weeks after starting capecitabine and 10 months after palliative RT. Imaging showed typical findings of radiation myelopathy. The patient was treated with corticosteroids and submitted to hyperbaric oxygen, with clinical improvement after 3 months. To the best of our knowledge, this is the first report of radiation recall myelitis associated with capecitabine.

Background Huntington's disease (HD) is an autosomal-dominant neurodegenerative disorder inducing motor, psychiatric changes and cognitive decline, characterized pathologically by striatal atrophy. Pathological changes in the extra-striatal structures, such as the substantia nigra (SN), and abnormalities in pre-synaptic striatal dopamine neurotransmission are also known to occur. Neuromelanin (NM)-sensitive magnetic resonance imaging (NM-MRI) is an innovative technique that was recently developed allowing the in vivo study of pathological changes in the dopaminergic neurons of the SN. Objective To investigate the SN MR signal in HD patients. Methods We performed a cross-sectional study using a specific T1-weighted MR sequence to visualize NM. The areas and signal intensity contrast ratios of the T1 hyperintense SN regions were obtained using a semi-automatic segmentation method. Results A total of 8 HD patients and 12 healthy subjects were evaluated. The SN area was markedly reduced in the HD group compared with the control group (p = 0.02), even after normalization of the SN area with the midbrain area and age correction (p = 0.01). There was a significant reduction in the intensity contrast ratio of the hyperintense SN areas to crus cerebri in HD patients comparing with controls (p = 0.04) after correction for age. Conclusions NM-sensitive MR techniques were used for the first time to study the SN in HD patients, showing loss of NM in this region, supporting the implication of dopaminergic neuronal changes in disease pathology. Future research needs to be conducted to evaluate the potential of SN area and intensity contrast as biomarkers for HD.

Introduction et objectifs Le faisceau corticospinal (FCS) peut etre selectivement atteint par des nombreuses pathologies : neurodegeneratives, oncologiques, toxiques/metaboliques, infectieuses, auto-immunes et genetiques. L’imagerie par resonance magnetique (IRM) est indispensable pour la reconnaissance du FCS, l’identification des anomalies de signal des faisceaux de substance blanche et le diagnostic differentiel des lesions du FCS. Il permet d’effectuer une evaluation precise du parenchyme cerebral et d’identifier des lesions associees qui peuvent aider dans le diagnostic differentiel d’un large spectre de maladies. Les objectifs sont de decrire, caracteriser et illustrer les aspects en IRM des lesions du FCS suivant la litterature ; illustrations et revision des cas cliniques de notre centre. Materiels et methodes Nous avons : –revise les caracteristiques des anomalies de signal en IRM du FCS decrite dans la litterature, concentre sur le diagnostique differentiel ; –fait une revision des lesions du FCS des cas cliniques de notre hopital. Resultats Les anomalies de signal du FCS en IRM peuvent etre dues a diverses maladies avec une prevalence importante de la sclerose laterale amyotrophique, primitive et de l’infection par le VIH. Les caracteristiques d’image peuvent aider enormement dans le diagnostic differentiel. Dans la majorite des cas nous relevons un hypersignal dans les ponderations avec un TR long du FCS (en partie ou en totalite). Dans notre hopital, nous avons identifie des cas de sclerose laterale amyotrophique, de leucodystrophie et d’infection par le VIH. L’imagerie par IRM a ete fondamentale pour l’etude etiologique. Conclusion Le FCS peut etre atteint par des nombreuses pathologies ayant un large diagnostique differentiel. Les alterations du FCS en IRM peuvent etre non specifiques mais avec des alterations associees peuvent aider amplement dans l’identification de l’etiologie et des differents traitements disponibles et definitions des pronostics.

IMPORTANCE: Parkinson disease (PD) manifests by motor and nonmotor symptoms, which may be preceded by mood disorders by more than a decade. Bipolar disorder (BD) is characterized by cyclic episodes of depression and mania. It is also suggested that dopamine might be relevant in the pathophysiology of BD. OBJECTIVE: To assess the association of BD with a later diagnosis of idiopathic PD. DATA SOURCES: An electronic literature search was performed of Cochrane Controlled Register of Trials, MEDLINE, Embase, and PsycINFO from database inception to May 2019 using the terms Parkinson disease, bipolar disorder, and mania, with no constraints applied. STUDY SELECTION: Studies that reported data on the likelihood of developing PD in BD vs non-BD populations were included. Two review authors independently conducted the study selection. DATA EXTRACTION AND SYNTHESIS: Two review authors independently extracted study data. Data were pooled using a random-effects model, results were abstracted as odds ratios and 95% CIs, and heterogeneity was reported as I(2). MAIN OUTCOME AND MEASURES: Odds ratios of PD. RESULTS: Seven studies were eligible for inclusion and included 4 374 211 participants overall. A previous diagnosis of BD increased the likelihood of a subsequent diagnosis of idiopathic PD (odds ratio, 3.35; 95% CI, 2.00-5.60; I(2) = 92%). A sensitivity analysis was performed by removing the studies that had a high risk of bias and also showed an increased risk of PD in people with BD (odds ratio, 3.21; 95% CI, 1.89-5.45; I(2) = 94%). Preplanned subgroup analyses according to study design and diagnostic certainty failed to show a significant effect. CONCLUSIONS AND RELEVANCE: This review suggests that patients with BD have a significantly increased risk of developing PD compared with the general population. Subgroup analyses suggested a possible overestimation in the magnitude of the associations. These findings highlight the probability that BD may be associated with a later development of PD and the importance of the differential diagnosis of parkinsonism features in people with BD.

© Springer-Verlag GmbH Germany, part of Springer Nature 2018, Purpose: This paper aims to analyze the contribution of mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) in the detection of microstructural abnormalities in amyotrophic lateral sclerosis (ALS) and to evaluate the degree of agreement between structural and functional changes through concomitant diffusion tensor imaging (DTI), transcranial magnetic stimulation (TMS), and clinical assessment. Methods: Fourteen patients with ALS and 11 healthy, age- and gender-matched controls were included. All participants underwent magnetic resonance imaging including DTI. TMS was additionally performed in ALS patients. Differences in the distribution of DTI-derived measures were assessed using tract-based spatial statistical (TBSS) and volume of interest (VOI) analyses. Correlations between clinical, imaging, and neurophysiological findings were also assessed through TBSS. Results: ALS patients showed a significant increase in AD and MD involving the corticospinal tract (CST) and the pre-frontal white matter in the right posterior limb of the internal capsule (p < 0.05) when compared to the control group using TBSS, confirmed by VOI analyses. VOI analyses also showed increased AD in the corpus callosum (p < 0.05) in ALS patients. Fractional anisotropy (FA) in the right CST correlated significantly with upper motor neuron (UMN) score (r = - 0.79, p < 0.05), and right abductor digiti minimi central motor conduction time was highly correlated with RD in the left posterior internal capsule (r = - 0.81, p < 0.05). No other significant correlation was found. Conclusion: MD, AD, and RD, besides FA, are able to further detect and characterize neurodegeneration in ALS. Furthermore, TMS and DTI appear to have a role as complementary diagnostic biomarkers of UMN dysfunction.

Background Specific MR sequences have been able to identify the loss of neuromelanin in the substantia nigra (SN) of early stage Parkinson's disease (PD) patients. Since this technique may have a significant impact in clinical patient management, easy and widely available imaging analysis is needed for routine use. Objective In this study we compared a quantitative analysis with a visual assessment of SN neuromelanin-sensitive MR images in early stage PD patients, in terms of pattern changes recognition and diagnostic accuracy. Methods The inclusion criteria were untreated "de novo" PD patients or a 2-5 year PD duration; in addition, age matched controls were enrolled. These were studied with a high-resolution T1-weighted MR imaging sequence at 3.0 Tesla to visualize neuromelanin. The primary outcome was the comparison of quantitative width measurement with visual assessment by experienced neuroradiologists of SN neuromelanin sensitive MR images for PD diagnosis. Results A total of 12 "de novo" PD patients, 10 PD patients with 2-5 year disease duration and 10 healthy controls were evaluated. We obtained a good accuracy in discriminating early-stage PD patients from controls using either a quantitative width measurement of the T1 high signal or a simple visual image inspection of the SN region. Conclusions Visual inspection of neuromelanin-sensitive MR images by experienced neuroradiologists provides comparable results to quantitative width measurement in the detection of early stage PD SN changes and may become a useful tool in clinical practice.

Objective The human nucleus accumbens (Acc) has become a target for deep brain stimulation (DBS) in some neuropsychiatric disorders. Nonetheless, even with the most recent advances in neuroimaging it remains difficult to accurately delineate the Acc and closely related subcortical structures, by conventional MRI sequences. It is our purpose to perform a MRI study of the human Acc and to determine whether there are reliable anatomical landmarks that enable the precise location and identification of the nucleus and its core/shell division. Methods For the Acc identification and delineation, based on anatomical landmarks, T1WI, T1IR and STIR 3T-MR images were acquired in 10 healthy volunteers. Additionally, 32-direction DTI was obtained for Acc segmentation. Seed masks for the Acc were generated with FreeSurfer and probabilistic tractography was performed using FSL. The probability of connectivity between the seed voxels and distinct brain areas was determined and subjected to k-means clustering analysis, defining 2 different regions. Results With conventional T1WI, the Acc borders are better defined through its surrounding anatomical structures. The DTI color-coded vector maps and IR sequences add further detail in the Acc identification and delineation. Additionally, using probabilistic tractography it is possible to segment the Acc into a core and shell division and establish its structural connectivity with different brain areas. Conclusions Advanced MRI techniques allow in vivo delineation and segmentation of the human Acc and represent an additional guiding tool in the precise and safe target definition for DBS.

Background Essential tremor (ET) is a very common movement disorder that has no diagnostic markers. Differentiation with Parkinson's disease (PD) can be clinically challenging in some cases, with a high rate of misdiagnosis. Magnetic resonance imaging (MRI) studies have been able to identify neuromelanin changes in the substantia nigra (SN) of PD patients, but they have thus far not been investigated in ET. In this study, we aimed to characterize neuromelanin-MR signal changes in ET and evaluate its diagnostic accuracy in the differential diagnosis with PD. Methods The inclusion criteria were patients with ET and untreated “de novo” PD patients; in addition, age-matched controls were enrolled. These were studied with a high-resolution T1-weighted MRI sequence at 3.0 Tesla to visualize neuromelanin. The primary outcomes were the area and width of the SN region with high signal. Results A total of 15 ET patients and 12 “de novo” PD patients were evaluated. The area and width of the T1 high signal in the SN region were markedly decreased in the PD group compared with the ET and age-matched controls, and a greater decrease was seen in the ventrolateral segment. The neuromelanin measures in the ET group, although slightly lower, were not significantly different from the healthy control group. We obtained a sensitivity of 66.7% and a specificity of 93.3% in discriminating ET from early-stage PD. Conclusions Neuromelanin-sensitive MRI techniques can discriminate ET from early-stage tremor-dominant PD and can be a useful clinical tool in the evaluation of tremor disorders. © 2015 International Parkinson and Movement Disorder Society

© 2017 – IOS Press and the authors. All rights reserved, Background: A specific T1-weighted magnetic resonance imaging (MRI) sequence has been shown to detect substantia nigra (SN) neuromelanin (NM) signal changes that accurately discriminate Parkinson's disease (PD) patients from controls, even in early disease stages. However, it is unclear what happens to these SN changes in later disease stages and if they can be a marker of disease progression. Objective: to investigate the pattern of SN-NM area loss and contrast ratio (CR) intensity changes in late-stage PD (LSPD) compared to earlier disease stages. Methods: A comparative cross-sectional study was performed, analyzing SN-NM MRI signal in LSPD (Schwab and England Activities of Daily Living Scale score 3), comparing this group with de novo, 2-5 year PD and controls. SN-NM signal area and CR values for the internal and lateral SN regions were obtained with semi-automated methods. Results: 13 LSPD, 12 de novo patients with PD, 10 PD patients with a 2-5 year disease duration, and 10 controls were included. NM signal area was significantly decreased in LSPD compared to de novo PD (P-value = 0.005; sensitivity: 75%; specificity 92% and AUC: 0.86). In the lateral SN region, a decrease in the CR was detected in all PD groups compared to controls; despite not reaching statistical significance, a slight increment was observed comparing LSPD to 2-5 year PD. NM signal area significantly correlated with HY (R = -0.37; P < 0.05) and Movement disorder Society Unified Parkinson's Disease Rating Scale part II (MDS-UPDRS) (R = -0.4; P < 0.05) while a weak correlation was found with MDS-UPDRS part III (R = -0.26; P: 0.1). Conclusion: SN area evaluated by NM-sensitive MRI may be a promising biomarker of nigral degeneration and disease progression in PD patients.

Dissertação de mestrado em Gestão de Projetos de Engenharia, A implementação das melhores práticas de gestão de projetos é um fator preponderante para o sucesso das empresas. Este destaque surgiu da necessidade das organizações responderem celeremente, eficientemente e de forma integrada aos desafios emergentes das suas rotinas diárias. A finalidade do trabalho de investigação focou-se em espelhar uma solução embrionária para a implementação das iniciativas de gestão de projetos na empresa. Como resultado desta investigação, foram identificadas as iniciativas de melhoria de gestão de projetos nas quais uma empresa do setor de engenharia e construção deve focar os seus esforços, tendo em consideração o seu contexto organizacional e o baixo nível de maturidade organizacional em gestão de projetos. Três dimensões foram consideradas para o desenvolvimento desta proposta: Pessoas e Conhecimento Organizacional; Processos, Ferramentas e Técnicas e Sistema Organizacional. Esta proposta envolve assim a integração do ciclo de vida de gestão de projetos com o ciclo de vida dos projetos da empresa em estudo, transversal a todas as áreas de conhecimento descritas pelo PMBoK, aliando as técnicas e ferramentas sugeridas pela revisão de literatura para uma melhor gestão dos projetos., The implementation of project management best practices is a preponderant factor for the success of companies. Organizations need to respond quickly, efficiently and in an integrated way to the challenges emerging from daily routines. The purpose of the research work focused on presenting a solution for the implementation of project management initiatives in the company. As an outcome, project management improvement initiatives in which an engineering and construction company should focus its efforts were identified, considering its organizational context and low level of organizational maturity in project management. Three dimensions were considered for the development of this proposal: People and Organizational Knowledge; Processes, Tools and Techniques and General Management System. This proposal thus involves the integration of the project management lifecycle with the life cycle of the projects of the company under study, transversal to all the areas of knowledge described by PMBoK, combining the techniques and tools suggested by the literature review for a better management of projects.

"BACKGROUND: There is scarce data on the level of handicap in Parkinson's disease (PD) and none in advanced stage PD. OBJECTIVE: To assess the handicap in advanced stage PD patients with disabling levodopa-induced motor complications selected to deep brain stimulation (DBS). METHODS: Data was prospectively recorded during routine evaluation for DBS. Handicap was measured using London Handicap Scale (LHS) (0 = maximal handicap; 1 = no handicap). Disease severity was evaluated using the Hoehn & Yahr scale and the UPDRS/MDS-UPDRS, during off and on after a supra-maximal dose of levodopa. Schwab and England Scale (S&E) was scored in off and on. Dyskinesias were scored using the modified Abnormal Involuntary Movement Scale (mAIMS). Results concern cross-sectional assessment before DBS. RESULTS: 100 PD patients (mean age 61 (±7.6); mean disease duration 12.20 (±4.6) years) were included. Median score of motor MDS-UPDRS was 54 in off and 25 in on. Mean total LHS score was 0.56 (±0.14). Patients were handicapped in several domains with a wide range of severity. Physical Independence and Social Integration were the most affected domains. Determinants of total LHS score were MDS-UPDRS part II off (β= -0.271; p = 0.020), S&E on (β= 0.264; p = 0.005) and off (β= 0.226; p = 0.020), and mAIMS on (β= -0.183; p = 0.042) scores (R2 = 29.6%). CONCLUSIONS: We were able to use handicap to measure overall health condition in advanced stage PD. Patients were moderately to highly handicapped and this was strongly determined by disability in ADL and dyskinesias. Change in handicap may be a good patient-centred outcome to assess efficiency of DBS." info:eu-repo/semantics/publishedVersion

A 39-year-old man developed transient expressive aphasia and headache following extreme breath-hold diving. He had performed around 30 breath-hold dives, lasting approximately 2 minutes, 30 meters deep, for 5 hours, with short surface intervals. Brain MRI (figure, A–C) revealed a left frontal white matter lesion, without restricted diffusion, contrast enhancement, or mass effect.

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 (

3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) lyase deficiency is a rare inborn error affecting leucine catabolism and ketogenesis, usually presenting in the neonatal period. Late forms of the disease have been detected in infancy and childhood, but not in adults. We report a case of HMG-CoA lyase deficiency with initial presentation in a 29-year-old adult with no prior history of the disease, which to our knowledge is the first case described with presentation at this age.

Background and purpose Magnetic resonance (MR) studies have demonstrated a significant reduction of neuromelanin in the substantia nigra (SN) of Parkinson's disease (PD) patients with high accuracy for differential diagnosis compared to non-PD controls and essential tremor. However, studies state that not knowing how paramagnetic effects of iron influence neuromelanin signal is a limitation. In this study a neuromelanin-sensitive MR sequence was combined with T2* relaxometry iron quantification analysis to study the SN of early-stage PD patients to investigate the correlation between these parameters. Methods The inclusion criteria were untreated de novo PD patients and a 2–5 year disease duration (early PD); in addition, age-matched controls were enrolled. These were studied at 3.0 T with a high-resolution T1-weighted MR sequence to visualize neuromelanin and a relaxometry sequence for iron quantification. The primary outcome was the correlation of the width of the neuromelanin high signal region and the T2* values in the lateral, central and medial segments of the SN. Results Very weak correlations of T2* values with neuromelanin width, positive for global and negative for the medial and lateral SN segments, were found in both PD groups and control subjects. The SN neuromelanin width was markedly reduced in the de novo and early PD groups compared with controls in all SN segments, but no significant difference in T2* values was found between the groups. Conclusions The SN neuromelanin signal does not have a significant correlation with iron content in PD patients or controls. The neuromelanin MR signal reduction in PD does not seem to be significantly influenced by paramagnetic iron effects.

Essential tremor (ET) is a very common movement disorder that has no diagnostic markers. Differentiation with Parkinson's disease (PD) can be clinically challenging in some cases, with a high rate of misdiagnosis. Magnetic resonance imaging (MRI) studies have been able to identify neuromelanin changes in the substantia nigra (SN) of PD patients, but they have thus far not been investigated in ET. In this study, we aimed to characterize neuromelanin-MR signal changes in ET and evaluate its diagnostic accuracy in the differential diagnosis with PD.The inclusion criteria were patients with ET and untreated "de novo" PD patients; in addition, age-matched controls were enrolled. These were studied with a high-resolution T1-weighted MRI sequence at 3.0 Tesla to visualize neuromelanin. The primary outcomes were the area and width of the SN region with high signal.A total of 15 ET patients and 12 "de novo" PD patients were evaluated. The area and width of the T1 high signal in the SN region were markedly decreased in the PD group compared with the ET and age-matched controls, and a greater decrease was seen in the ventrolateral segment. The neuromelanin measures in the ET group, although slightly lower, were not significantly different from the healthy control group. We obtained a sensitivity of 66.7% and a specificity of 93.3% in discriminating ET from early-stage PD.Neuromelanin-sensitive MRI techniques can discriminate ET from early-stage tremor-dominant PD and can be a useful clinical tool in the evaluation of tremor disorders. © 2015 International Parkinson and Movement Disorder Society.